Help For Hearing Loss Sufferers
Speech and language processing are largely intact in older adults under normal conditions, although processing time may be somewhat slower than in young adults. In fact, there is evidence that discourse skills actually improve with age. Older people often tell well-structured elaborate narratives that are judged by others to be more interesting than those told by young 46 . They usually have more extensive vocabularies and although they exhibit the occasional word-finding difficulty, older adults are easily able to provide circumlocutions to mask the problem. They are skilled conversationalists and appear to have few difficulties in processing ongoing speech. As noted above, however, some older adults have hearing loss and so, in conversational settings, may be required to interpret a weak or distorted acoustic signal. Even under these conditions, older people seem able to maintain good levels of comprehension by effectively using context to interpret the message 47 . Nevertheless,...
Other heredofamilial optic neuropathies are very uncommon. They are encountered primarily in cases in which additional, and unexpected, deficits develop. Behr's recessive optic atrophy affects young males under 10 years of age, accompanied by mild cognitive impairment, spasticity, ataxia, and muscular hypertonicity. A rare form of reces-sively inherited optic neuropathy is found accompanied by type I diabetes, deafness, and diabetes insipidus (hence DIDMOAD diabetes insipidus, diabetes mellitus, optic atrophy, and deafness - or Wolfram's syndrome). Optic atrophy also commonly develops in conjunction with various forms of spino- or olivocerebellar degenerations, such as Friedreich's ataxia.
The human white or ABCG subfamily consists of five fully cloned genes (ABCG1, ABCG2, ABCG4, ABCG5, and ABCG8) and one gene so far only found in rodents (ABCG3) 22 . The ABCGs are thought to dimerize to form active membrane transporters. Among the half-size molecules ABCG proteins have a peculiar domain organization characterized by a nucleotide binding domain (ATP binding cassette) at the N-terminus followed by six transmembrane-spanning domains (Tab. 3.1 and Fig. 3.1). The founding member of this group, ABCG1, was independently described by Chen et al. and Croop et al. as the human homolog of the Dro-sophila white gene 112, 113 and its genomic organization, including the promoter region, has been described recently 114, 115 . Earlier indications linked ABCG1 with the congenital recessive deafness (DFNB10) syndrome, based on its chromosomal localization on chromosome 21q22.3 116 . However, a recent report 117 has excluded ABCG1 along with five other known genes as candidates for...
Mechanisms of the severe ototoxicity associated with aminoglycoside use are only partially known, but circumstantial data suggest that aminoglycoside ototoxicity results from unintended interactions with human mitochondrial ribosomes. (1) Reports of several pedigrees exist, which correlate hypersensitivity to aminoglycosides with a single polymorphism in the mitochondrial genome (A1555G) (72-76). This polymorphism conspicuously occurs in the A-site of 12S rRNA, which is analogous to the gentamicin neomycin binding site in bacterial ribosomes. It is speculated that this polymorphism makes the A-site of (mitochondrial) look more like the A-site of 16S rRNA (bacterial) (Fig. 6.5). (2) The phenotype of sensoneural hearing loss or deafness due to aminoglycoside toxicity is the same as that found in most mitochondrial en-cephalomyopathies,suggesting mitochondrial involvement (77). (3) Mitochondrial DNA from individuals with the A1555G polymorphism can be co-transfected into p HeLa cells,...
Beals acknowledged that he taught himself not to hear his wife going on and on about his habits. He did not want unpleasantness, and it was simpler just to ignore her. In other words, he negated her very presence. This could be regarded as an extreme illustration of indirect and masked communication. Indirect, because Mrs. Beals had only a vague notion that his loss of hearing was specifically directed at her, and masked, because his message was totally lost on Mrs. Beals. She did not recognize his phony deafness as anything other than a plausible affliction of advancing years.
The disease affects neonates, infants, and juveniles. Coarse facial features and skeletal anomalies resembling mucopolysaccharidosis, corneal opacity, deafness, and mental retardation are characteristic clinical features. The pathology combines the features of metachromatic leukodystrophy with those of mucopolysaccharidosis.
Myoclonus epilepsy with ragged red fibers (MERRF) affects children and adults. Cardinal clinical manifestations include short stature, seizures, polymyoclonus, optic atrophy, sensory-neuronal hearing loss, cerebellar ataxia, peripheral neuropathy, and myopathy. The disease is maternally transmitted and is associated with point mutations in tRNA gene.
In 1988, Harding and colleagues (44) and Wallace et al. (45) reported the first mitochondrial mutations causing human disease. Since then, many other mi-tochondrial diseases caused by mitochondrial point mutations or multiple mito-chondrial deletions have been identified (46). There are a number of typical presentations of mitochondrial disease in humans, including (1) Kearns-Sayre syndrome, characterized by ptosis, ophthalmoplegia, retinitis pigmentosa, hearing loss, cardiac conduction defects, short stature, and elevated cerebrospinal fluid protein (2) mitochondrial encephalopathy with lactic acidosis and strokes (MELAS) (3) myoclonic epilepsy with ragged red fibers (MERRF) (4) Leber hereditary optic neuropathy (LHON) with sudden unilateral or bilateral painless central visual loss and (5) Leigh syndrome, or subacute necrotizing en-cephalomyopathy. The pleiotropic manifestations of mitochondrial disease may include, for example, diabetes mellitus, hearing loss, bone marrow aplasia,...
This analysis has implications for teaching and learning. When most people learn from instruction but a few do not, educators, psychologists, and parents often blame the poor student, confused client, or stubborn child. They see the failure to learn as a deficiency of the person rather than as a problem of contingencies of reinforcement (called blaming the victim, Shaver, 1985). The ones that fail to learn are said to be learning disabled, low in intelligence, dim-witted, and so on. Of course, some people and animals may have neurological and or sensory impairment (e.g., color blindness, deafness, organic brain damage) that contributes to their poor performance. Nonetheless, defective contingencies of reinforcement also may contribute to, or exclusively produce, problems of discrimination and learning. In the case of the apparently dumb pigeon, the fault was caused entirely by adventitious reinforcement of responding during extinction. A small change in the contingencies of...
The long QT syndrome (LQTS) is an inherited disease characterized by an abnormally prolonged ventricular repolarization that creates a vulnerable substrate for the development of life-threatening arrhythmias (Schwartz et al. 2000b). Two phenotypic variants havebeen described the autosomal-dominant Romano-Ward Syndrome (RW) and the autosomal-recessive Jervell-Lange-Nielsen syndrome (JLN) in the latter, the cardiac phenotype is associated with sensorineural deafness. The JLN syndrome is a recessive disease allelic to LQT1 and LQT5, in which homozygous or compound heterozygous mutations recapitulate the pheno-type prolonged Q-T interval, cardiac arrhythmias, and deafness (Neyroud et al. 1997 Schulze-Bahr et al. 1997).
A patient with a completely nonfunctioning cochlea is referred to as having nerve deafness . In fact, there is evidence that in many cases the nerves may be intact, while the receptor cells and organ of Corti are defective. For such patients, a goal is to restore hearing with cochlear electrode implants to stimulate the nerve endings directly. Significant progress has been made. However, despite electrode stimulation of nerves at the correct place along the cochlea for a high frequency, the perception of high frequency has not been achieved. So, although substantial advance in cochlear physiology has been made in the recent past, several such waves of progress may be needed to adequately understand the functioning of the cochlea.
A 15-year-old member of the high school marching band comes to your office for evaluation of hearing loss. He had multiple ear infections as an infant and toddler, and had to have myringotomy tubes inserted in his ears. Additionally, he suffers from many allergies. His hearing is diminished in the right ear. When you place a vibrating tuning fork on the top of his head, the sound lateralizes to the right ear. The name of this test is
AMM occurs primarily in older patients. Nearly two thirds of the cases occur between the ages of 50 and 70, about equally in men and in women. Symptoms and signs depend upon the stage of disease when the patient is first encountered. Most often, symptoms are related to anemia and or an enlarged spleen. Otosclerosis, which can be a presenting symptom in advanced cases, causes deafness in about 10 of patients.
Summary A 75-year-old man who presents with loss of speech discrimination and complains of difficulty understanding speech and conversation in noisy areas most likely has presbycusis. Presbycusis is age-related sensorineural hearing loss typically associated with both selective high-frequency loss and difficulty with speech discrimination. Physical examination of the ears in patients with presbycusis is normal. Next step Presbycusis is a diagnosis of exclusion. Hearing aids are underused in presbycusis but are potentially beneficial for most types of hearing loss, including sensorineural hearing loss. Consequently, referral to an audiologist for testing and consideration of amplification with a hearing aid may be an important next step.
Lafora disease with myoclonic epilepsy, a rare autosomal recessive disorder of juveniles, is caused by mutations in the gene EPM2A, located on chromosome 6. The disease presents with tonic-clonic seizures, widespread stimulus-induced myoclonic jerks, hearing impairment, ataxia, and mental regression. Over several years, the disease progresses to total disability. Death usually occurs in the third decade of life.
2 The nasopharyngeal tonsils (adenoids) are prominent in children but usually undergo atrophy after puberty. When chronically inflamed they may all but fill the nasopharynx, causing mouth-breathing and also, by blocking the auditory tube, deafness and middle ear infection.
As the child grows, the ease and techniques of examining the ears and testing the hearing approach the methods used for adults. There are no ear abnormalities or variations of normal unique to this age group. Of course, parents of older children routinely refer to the normal condition of selective deafness, defined as choosing to hear what the child wishes to hear.
This disease presents in adulthood, but earlier onsets are not uncommon. Cardinal clinical features are slowly progressive sensorimotor neuropathy, retinitis pigmentosa, optic atrophy, ichthyosis of the skin and, later in the course, cerebellar ataxia. Additional features are cardiomyopathy, anosmia, and neuronal hearing impairment. Skeletal anomalies and cutaneous changes may occur.
Phototherapy is often the treatment of choice when bilirubin levels exceed 10 mg dL since kernicterus may occur at levels approaching 20 mg dL. Phototherapy is a method of treating neonatal hyperbilirubinemia in which the baby is placed periodically under a light source emitting 450-nm wavelength light. Light diffuses through layers of skin and converts unconjugated bilirubin to stable water-soluble forms that can be excreted. The baby's eyes are protected during this process from harmful ultraviolet (UV) and near-UV rays. Kernicterus is a condition in which brain cell nuclei stain yellow and become damaged due to bilirubin or other molecules. High levels of bilirubin are less likely to cause brain damage in adults due to the natural barrier in the brain, called the blood-brain barrier. Ker-nicterus typically occurs at bilirubin levels greater than 20 mg dL in infants due to their immature blood-brain barrier. It may result in cerebral palsy, deafness, or mental retardation.
Childhood, juvenile, and adult adrenoleukodystrophies are characterized by mental regression progressing to dementia, motor disorders, visual and sensorineuronal hearing loss, and seizures culminating in a terminal vegetative state. Death occurs 2 to 3 years after onset in childhood cases. The clinical course is longer in juvenile and adult cases.
Joint position and vibration, and loss of tendon reflexes. During the course of the disease, which averages from 20 to 25 years, optic atrophy, sensorineural hearing impairment, weakness and spasticity in lower extremities, Babinski sign, and dysarthria develop. Characteristic systemic manifestations include skeletal anomalies with scoliosis and pes cavus, hypertrophic cardiomyop-athy, and occasional diabetes mellitus. FA, an autosomal recessive disease, is caused by an expansion of guanine-adenine-adenine (GAA) repeats in the frataxin gene on chromosome 9. Expansion of the repeats is from 90 to more than 100. The normal number of repeats is 6 to 27.
Kearns-Sayre Syndrome (KSS), with onset in adolescence, is distinguished by progressive external ophthal-moplegia, pigmentary retinopathy, sensorineuronal hearing loss, cerebellar ataxia, heart block, mental regression, myopathy, and elevated CSF protein. Neuro-imaging shows white matter hyperintensities on T2-weighted MRI and calcifications in the basal ganglia
Cockayne syndrome, a rare autosomal recessive disorder, is caused by mutations in either the CSA or CSB gene. The disease manifests with dwarfism microcephaly facial dysmorphism with prognathism, malformed large ears, enophthalmos, and beaked nose long extremities and sensitivity to sunlight. Neurologic abnormalities are mental retardation, sensorineuronal hearing impairment, optic atrophy and pigmentary retinal degeneration, myoclonus, and pyramidal and extrapyramidal symptoms and signs.
Adverse effects are uncommon, apart from excess of therapeutic effect (electrolyte disturbance and hypotension due to low plasma volume) and those mentioned in the general account for diuretics (below). They include nausea, pancreatitis and, rarely, deafness which is usually transient and associated with rapid i.v. injection in renal failure. NSAIDs, notably indomethacin, reduce frusemide-induced diuresis probably by inhibiting the formation of vasodilator prostaglandins in the kidney. Bumetanide, piretanide and ethacrynic acid are similar to frusemide. Torasemide is also similar, but has also been demonstrated to be an effective antihypertensive agent at lower (non-natriuretic) doses (2.5-5 mg d) than those used for oedema (5-40 mg). Ethacrynic acid is less widely used as it is more prone to cause adverse effects, especially nausea and deafness.
Middle ear to the cochlea, a part of the inner ear. The cochlea senses and codes the vibrations, and nerve impulses are sent to the brain through the cochlear nerve. The first part of this pathway from the external ear through the middle ear is known as the conductive phase, and a disorder here causes conductive hearing loss. The second part of the pathway, involving the cochlea and the cochlear nerve, is called the sensorineural phase a disorder here causes sensorineural hearing loss. Acuity of hearing, like that of vision, usually diminishes with age. Early losses, which start in young adulthood, involve primarily the high-pitched sounds beyond the range of human speech and have relatively little functional significance. Gradually, however, loss extends to sounds in the middle and lower ranges. When a person fails to catch the upper tones of words while hearing the lower ones, words sound distorted and are difficult to understand, especially in noisy environments. Hearing loss...
Hoarseness (often but not always present with CN10 lesions) and difficulty swallowing. 5-2 A patient chronically ingesting full doses of streptomycin, quinine, and aspirin began complaining of bilateral hearing loss. Why Ans. All these agents can damage the auditory nerve. 5-3 Draw a single unilateral lesion accounting for the following symptoms and signs. G. Progressive left-sided deafness and left lower motor neuron facial paralysis, absent left-sided cold caloric responses. K. Left-sided headache, total paralysis left side of face with vertigo and left-sided hearing loss no other neurological deficit. Ans. Possible left-sided cerebellopontine angle tumor (outside the brain stem). 5-15 If you stick a finger in your left ear and touch a tuning fork to the forehead midline (or simply hum), which ear hears the sound loudest Ans. The left ear, for you have simulated a conductive loss on the left, in which bone conduction is accentuated. Ear wax occlusion and damage to the tympanic...
Patients with LQTS are usually identified by QT prolongation on the ECG during clinical evaluation of unexplained syncope, as part of a family study when one family member has been identified with the syndrome, or in the investigation of patients with congenital neural deafness. The first family with LQTS was reported in 1957 by Jervell and Lange-Nielsen and was thought to be
Increased potassium excretion and the elevation of plasma uric acid levels, as was observed with the thiazides, are also seen with the loop diuretics. These diuretics also increase calcium and magnesium excretion. The calciuric action of these agents has led to their use in symptomatic hypercalcemia (301).Many of the hypersensitivity and metabolic disorders seen with the thiazides are also seen with loop diuretics. The development cf transient or permanent deafness is a serious but rare complication observed with this class of agents. It is believed to arise from the changes in electrolyte composition of the endolymph (302). It usually occurs when blood levels of these drugs are very high.
Common neurologic symptoms of HVS include headaches, nausea, vertigo, and dizziness, which rarely can progress to coma.114'35 36 Some patients with HVS can show sensorineural hearing loss.37 In most cases, neurologic symptoms usually appear at higher viscosity levels (i.e., 4 cp), though early appearance before retinal findings at lower serum viscosity levels (i.e., 2-3 cp) has also been reported.18
Excessive use of sodium nitroprusside for severe hypertension.9 The symptoms of acute poisoning are due to tissue anoxia, with dizziness, palpitations, a feeling of chest constriction and anxiety characteristically the breath smells of bitter almonds. In more severe cases there is acidosis and coma. Inhaled hydrogen cyanide may lead to death within minutes but when it is ingested as the salt several hours may elapse before the patient is seriously ill. Chronic exposure damages the nervous system causing peripheral neuropathy, optic atrophy and nerve deafness.
The most frequent and common form of KD is the infantile form, which begins in the first 6 mo of life and rapidly progresses leading to death before the child reaches the age of 2. Children with KD present rapid psychomotor regression, generalized rigidity, and peripheral neuropathy they subsequently develop optic atrophy, deafness, and cachexia. Increased proteins in the cerebrospinal fluid is a constant finding cell count is usually normal. Nerve conduction velocity is always abnormal.
By the second year of life, hypotonia has given way to spas-ticity with tonic extensor spasms. Cortical blindness and optic atrophy develop. Sensorineural hearing loss may be present with developmental malformations of the organ of Corti (116). Macrocephaly is a prominent finding at this stage. The patient may have myoclonic or other seizures and choreoathetosis. Autonomic crises may occur with vomiting, temperature instability and vasomotor disturbances. A vegetative state develops and affected infants generally die in early childhood.
Ototoxicity is related to the total dose of cisplatin, is permanent, and occurs in 20-40 of patients. It begins as a high-frequency loss and progresses to involve frequencies in the speech range with continued exposure to the drug. The concurrent exposure to ototoxic environmental factors (e.g., rock concerts, airplanes, jack hammers) by adolescents and young adults may increase their risk of hearing loss. Appropriate anticipatory guidance and monitoring of audiograms may lessen the impact of ototoxicity. The delivery of only three cycles of cisplatin, when appropriate, will decrease the incidence of severe toxicity.
This group of rare lysosomal disorders results from deficiencies of enzymes involved in glycoprotein degradation. Glycoproteins consist of oligosaccharide chains attached to a protein core. Defective degradation of glycoproteins leads to the neurovisceral storage of oli-gosaccharides, glycopeptides, and glycolipids, and the urinary excretion of abnormal oligosaccharides. The group encompasses the mannosidosis, the fucosidoses, aspartyl-glycosaminuria, and Schindler's disease. The diseases affect neonates, infants, and young children, but juvenile and adult forms also occur. The clinical expression, except for the Schindler's disease, resembles that of Hurler's disease psychomotor retardation, facial and skeletal abnormalities, corneal opacity, deafness,
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Hearing Aids Inside Out
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