Help For Hearing Loss Sufferers

Hearing Aids Inside Out

Hearing Aids Inside Out

Have you recently experienced hearing loss? Most probably you need hearing aids, but don't know much about them. To learn everything you need to know about hearing aids, read the eBook, Hearing Aids Inside Out. The book comprises 113 pages of excellent content utterly free of technical jargon, written in simple language, and in a flowing style that can easily be read and understood by all.

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Breaking The Sound Barriers

Living with a deaf child is one of the hardest things that you will ever deal with in your life; the barriers to their learning and potential happiness can seem like you will never cross them. However, it is easier than you thing to learn how to communicate with your deaf child on a level that you both are able to understand. This ebook guide is designed to teach you how to truly communicate with your child so that it minimizes frustration for both of you, and allows you child to learn at a much higher level than almost anyone would have believed. You will also hear the success stories of deaf children that have gone on to achieve amazing success in their lives. Having a deaf child is a blessing, not a curse. This guide will show you how best to love and care for your child!

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Non Syndromic Deafness Cx26

The most common inherited sensory disorder is deafness. It is commonly classified into syndromic deafness, which is associated with other symptoms, and non-syndromic deafness, in which severe hearing defects are found in the absence of other symptoms. About 70 of all cases of prelingual deafness are non-syndromic. The disorder shows marked genetic heterogeneity, but in some families it maps to chromosome 13q11-12, the location of the gene encoding connexin 26 (Kelsell et al., 1997). Analysis of these families has revealed over 60 mutations in Cx26 that result in either dominant or recessive forms of non-syndromic deafness. The most common mutation is a deletion of the glycine residue at position 30 (G30), which results in the introduction of a premature stop codon and is expected to produce a non-functional protein (Denoyelle et al., 1997). This mutation is inherited in a recessive fashion and since heterozygotes have normal hearing, or only suffer mild high-frequency hearing loss in...

Mutations in KCNQ1 and minK may cause deafness

The Role Stria Vascularis Hearing

Expression of KCNQ1 and minK is not limited to the heart. Both are also expressed in the stria vascularis of the inner ear. This may explain why patients with recessive mutations in KCNQ1 (JLN syndrome) suffer from profound congenital deafness. Abnormalities in the C terminus of KCNQ1 have been found in some of these patients (Fig. 6.10). In two families, for example, a homozygous deletion of 7-bp and an 8-bp insertion at the same site was identified (Neyroud et al, 1997). This produced a frameshift at residue 415 and truncation of the protein close to the end of the normal coding region. A number of missense mutations have also been described (eg., W305S). The dominant negative effect of the JLN mutations is much less than that observed for the RW mutations, and correlates with the recessive mode of inheritance of JLN syndrome. Individuals homozygous for mutations in minK also suffer from deafness. Why do recessive KCNQ1 and minK mutations lead to deafness KCNQ1 is expressed in the...

Hearing Impairments

Auditory Cortex Pitch Tone Frequency

There are two major categories of deafness (1) conduction deafness, in which the transmission of sound waves through the middle ear to the oval window is impaired, and (2) sensorineural, or perceptive, deafness, in which the transmission of nerve impulses anywhere from the cochlea to the auditory cortex is impaired. Conduction deafness can be caused by middle-ear damage from otitis media or otosclerosis (discussed in the previous clinical applications box, p. 257). Sensorineural deafness may result from a wide variety of pathological processes and from exposure to extremely loud sounds. Unfortunately, the hair cells in the inner ears of mammals cannot regenerate once they are destroyed. Experiments have shown, however, that the hair cells of reptiles and birds can regenerate by cell division when they are damaged. Scientists are currently trying to determine if mammalian sensory hair cells might be made to respond in a similar fashion. Conduction deafness impairs hearing at all sound...

Hearing Loss

Hearing impairment may result from disease, injury, or developmental problems that affect the ear itself or any nervous pathways concerned with the sense of hearing. Sensorineural hearing loss results from damage to the eighth cranial nerve or to central auditory pathways. Heredity, toxins, exposure to loud noises, and the aging process are possible causes for this type of hearing loss. It may range from inability to hear certain frequencies of sound to a complete loss of hearing (deafness). People with extreme hearing loss that originates in the inner ear may benefit from a cochlear implant. This prosthesis stimulates the cochlear nerve directly, bypassing the receptor cells of the inner ear, and may allow the recipient to hear medium to loud sounds. Conductive hearing loss results from blockage in sound transmission to the inner ear. Causes include obstruction, severe infection, or fixation of the middle ear ossicles. Often the conditions that cause conductive hearing loss can be...

Infantile Malignant Osteopetrosis Marble Bone Disease

The marrow space is progressively obliterated by excessive osseous growth. The difficulty in obtaining marrow by aspiration is a diagnostic clue. Radiologic changes are characteristic and diagnostic, consisting of generalized osteosclerosis. The cranial foramina progressively narrow, resulting in blindness due to optic atrophy, deafness, and other cranial nerve lesions.


The aminoglycosides are contraindicated in patients with hypersensitivity to aminoglycosides. The amino-glycosides should not be given to patients requiring long-term therapy because of the potential for ototoxic-ity and nephrotoxicity. One exception is the use of streptomycin for long-term management of tuberculosis. These drugs are contraindicated in patients with preexisting hearing loss, myasthenia gravis, parkinson-ism, and during lactation or pregnancy. Neomycin, amikacin, gentamicin, kanamycin, netilmicin, and tobramycin are Pregnancy Category D drugs the remainder are Category C.

Restoration of Hearing

From that time on there have been attempts to transfer sound information directly to the auditory nerve or to the brainstem - bypassing the middle ear and even the cochlea as the physiological receivers and transmitters that are damaged in a variety of pathological conditions leading to profound deafness. The result of these endeavours was a device that could receive sound stimuli via a microphone and transform the acoustic characteristics into a series of time-distributed and intensity-modulated electrical impulses. With multiple electrodes on silicone carriers passed into the cochlea to contact the remaining nerve fibres (neurites) replacing the biological receptor cells (less refined than in nature, with a maximum of 23 electrodes today) in a frequency-specific order from the basal to the apical turns (cochlear implant) or onto the cochlear nucleus at the brainstem when the auditory nerve too was destroyed, these impulses were conveyed to the auditory pathway which ultimately...

Stateoftheart Trends 331 Hearing

Technologies for telemetric (wireless) testing and tuning of the implant have been developed that allow remote checking of the response of individual electrode channels (Kiss et al. 2003 Battmer et al. 2004). With appropriate tuning and training, speech perception can still improve even 5-10 years after implantation (Beadle et al. 2005b). In fact, the CI today is so advanced that some patients are fitted with a conventional hearing aid to improve hearing remnants on the contralateral side (Holt et al. 2005 Luntz et al. Up to now (2005) more than 400 patients with bilateral loss of hearing nerve function have received an ABI. Even though the temporal resolution and the dynamic features of the ABI are very similar to the cochlear implant (Shannon 1989 Shannon and Otto 1990), the clinical results with respect to speech comprehension did not meet early expectations and still do not significantly exceed the performance of single channel CIs - except in patients where the cause of deafness...

Pressureequalizing tubes in infancy

Ongoing controversy exists as to the best management of these young children with recurrent otitis and OME. Surgical treatment consists of myringotomy with insertion of a small tube through the tympanic membrane. It is the most common surgical procedure among infants and young children 27 . During the first 3 years of life the overall prevalence is 21 per 1000 children in the USA 28 . The primary goal of PE tube insertion is to remove middle ear effusion, prevent fluid accumulation and thus restore hearing, reduce recurrence of infections and prevent developmental delays in speech, language and cognition. The complications and sequelae of tympanostomy tube insertion have been examined by various studies, including one meta-analysis 22,29,30 . They include ear drum perforation, altered membrane appearance or tympanosclerosis (51 ), otorrhea (risk of 13 ), and cholesteatoma (rare, occurring 10 to 20 years later). While these complications may be noted in children with OME who do not...

Clinical and Endoscopic Findings

Even though painless facial and skull deformities are the most frequently observed signs, symptoms such as nasal obstruction, headache, epistaxis, anosmia, loosening of teeth, facial paralysis, hearing loss, trigeminal neuralgia-like pain, and recurrent rhinosinusitis due to drainage impairment may develop (Bollen et al. 1990 Camilleri 1991 Ferguson 1994 Slootweg et al. 1994 Wenig et al. 1995 Redaelli De Zinis et al. 1996 Chong and Tang 1997 Commins et al. 1998 Muraoka et al. 2001 Cheng et al. 2002). Diplopia, proptosis, loss of visual acuity due to optic nerve compression, epiphora, limitation of ocular motility are other important symptoms and signs indicating an involvement of the orbit and or of the lacrymal pathways (Moore et al. 1985 Osguthorpe and Gudeman 1987 Johnson et al. 1991 Slootweg et al. 1994 Wenig et al. 1995 Redaelli De Zinis et al. 1996). Since both diseases display a submucosal pattern of growth, nasal endoscopy is often negative or shows a lesion covered by intact...

And Laboratory Investigations

Most children with Hurler syndrome manage to walk, but develop only limited language skills. They have a characteristic appearance. Prominent features are relative macrocephaly, a prominent forehead, coarse facial features, hypertelorism, flat nasal bridge, prominent bushy eyebrows, thick and dry hair, hir-sutism, thick skin, enlarged tongue, hypertrophic gums, short and broad hands with stubby fingers, short and broad feet, exaggerated lumbar lordosis, thoracic kyphosis, and a protuberant abdomen, frequently with umbilical and inguinal hernias. On physical examination hepatosplenomegaly is found. Patients experience increasing joint stiffness and limitation of joint mobility, which may begin in early infancy. The preferentially affected joints are shoulders, fingers, and wrists. Deformities become apparent with claw hands and flexion contractures of elbows and knees. Vision becomes impaired due to progressive corneal clouding. Glaucoma, optic atrophy, and pigmentary retinal...

Circulatory Systems Of Lesser Volume

The bulbus oculi (eyeball) and the inner ear are fluid-filled hollow organs. Such organs have their own internal circulatory systems. In the case of the bulbus oculi, the fluid is the aqueous humor. In the case of the inner ear, the fluid is the endolymph perilymph. In such cases, the fluids are produced from fluids of arterial vessels and then are picked up by venous vessels. Should the drainage pattern be interrupted, fluids will accumulate and cause increased pressure within the hollow organ. The increased pressure will interfere with the organ functions examples are glaucoma of the eye and deafness of the ear.

Method of Computer Aided Adjustment of Hearing Aids

Widely-used systems for adjusting hearing aids allow for setting optimum parameters of a hearing aid, which usually does not mean that the optimum characteristics of hearing loss correction are obtained. The idea of a multimedia system for adjusting hearing aids (developed within the scope of a Ph.D. thesis by Piotr Suchomski 12 ) was to create a computer utility allowing for unrestrained shaping of the characteristics of the prospective hearing aid which would optimally compensate for the given hearing damage. Since in a general case the problem of adjusting a hearing aid can be boiled down to the problem of adjusting the wide dynamics of speech to the narrowed dynamics of impaired hearing, the presented system focusses mainly on determining the characteristics of impaired hearing and then on deriving the dynamics of the sought-after hearing aid that would compensate for the damage. This is, however, a simplification, because in a real hearing aid the quality of hearing loss...

Hearing training utilizing speechinnoise signals

The presented system utilizes a special module for converting results of loudness scaling into hearing dynamics. It uses fuzzy processing to map the results of scaling derived from the categories of the loudness sensation evaluation onto an objective scale of input sound levels, expressed in decibels. The category scale is represented by 7 fuzzy sets described by membership functions. These membership functions have been derived on the basis of statistical analysis of loudness scaling for 20 persons with no hearing impairments. The output of the fuzzy logic system is described by 13 membership functions which represent the differences between any given result of scaling and analogous results for unimpaired hearing 9 , 11 , 10 . Analysis of a typical plot of LGOB test results reveals that in between the seven categories of loudness sensation evaluations, one can define six differences which point to hearing loss (area below the LGOB curve for regular hearing) and six differences which...

External Auditory Meatus

The external auditory meatus develops from the dorsal portion of the first pharyngeal cleft (Fig. 16.7A). At the beginning of the third month, epithelial cells at the bottom of the meatus proliferate, forming a solid epithelial plate, the meatal plug (Fig. 16.7B). In the seventh month, this plug dissolves and the epithelial lining of the floor of the meatus participates in formation of the definitive eardrum. Occasionally the meatal plug persists until birth, resulting in congenital deafness.

Clinical Correlates

Deafness and External Ear Abnormalities Congenital deafness, usually associated with deaf-mutism, may be caused by abnormal development of the membranous and bony labyrinths or by malformations of the auditory ossicles and eardrum. In the most extreme cases the tympanic cavity and external meatus are absent. Most forms of congenital deafness are caused by genetic factors, but environmental factors may also interfere with normal development of the internal and middle ear. Rubella virus, affecting the embryo in the seventh or eighth week, may cause severe damage to the organ of Corti. It has also been suggested that poliomyelitis, erythroblastosis fetalis, diabetes, hypothyroidism, and toxoplasmosis can cause congenital deafness.

FDA and CDC collaboration on epidemiologic investigations

The epidemiological interpretation of the available data was complicated by several predisposing factors for meningitis in the cochlear implant population. First, certain congenital inner ear malformations, such as the Mondini malformation, are associated with cerebrospinal fluid (CSF) fistulae and recurrent episodes of meningitis, even without cochlear implantation or other otologic surgery 2 . Second, meningitis is the primary etiology of deafness for a significant number of adult and pediatric implant recipients, and it is well established that the risk for contracting meningitis is greater for an individual who has a prior history of this serious infection 3 . Third, there has been a strong trend toward implanting children at an earlier age, based on data showing improved long-term functional outcomes 4 . By the year 2000, one manufacturer had received FDA approval for use in children as young as 12 months. The incidence of meningitis is very high in this very young age group 5...

Gap Junction Channels

Gap junctions provide a means of coordinating the activity of adjacent cells or groups of cells. In excitable cells, gap junctions act as electrical synapses, enabling the fast transmission of electrical impulses between cells. They are important in synchronizing the electrical activity (and hence contraction) of cardiac and smooth muscle and the output of certain neuronal circuits, and their ability to relay information rapidly between pre- and postsynaptic neurones is utilised in escape behaviours such as the tail flip of the crayfish. They also synchronise exocytosis from endocrine cells, such as the islets of Langerhans. In non-excitable cells, the function of gap junctions is to permit the exchange of nutrients and regulatory signals between cells, such as intermediary metabolites and second messengers (Ca2+, cyclic AMP and IP3 are all permeant). They are involved in buffering the extracellular K+ concentration in the brain the local increase in K+ which results from neuronal...

Infertility in Mice Cx37

We have seen that gap junction channels form a pathway between the cytoplasm of adjacent cells. They are composed of two hemichannels (connexons), each of which is made up of six subunits (connexins). Multiple types of connexins exist and gap junctions may be formed from identical or different types of connexin. Mutations in one type of connexin, Cx32, produce CMTX, a disease in which the peripheral nerves degenerate. This appears to result because the gap junctions in the Schwann cells which surround the axons are no longer functional. Gross cardiac malformations result from mutations in Cx43 and congenital deafness from mutations in Cx26.

And Mitochondrial Leukoencephalopathies

Pyruvate Dehydrogenase Deficiency Mri

Although Leigh syndrome is a multisystem disorder, the clinical picture is dominated by signs of CNS dysfunction. In patients with neonatal and infantile onset, frequent signs are respiratory problems (irregular respiration, apnea, sighing, and hyperventilation), ocular abnormalities (strabismus, bizarre eye movements, external ophthalmoplegia, ptosis, optic atrophy, nystagmus, loss of vision, impaired pupillary reaction, retinal pigmentary degeneration), hypoto-nia, pyramidal signs (spastic paresis, hyperreflexia, extensor plantar reflexes), weakness, easy fatigability, and feeding problems (anorexia, difficulty in swallowing or sucking, vomiting, weight loss, and retarded growth). Episodes of lethargy, seizures, deafness, renal tubular dysfunction, and cardiac problems (car-diomyopathy and disturbances of cardiac rhythm with periods of tachycardia and bradycardia) may also be present. The same problems are frequent in later-onset forms of the disease, in addition to mental and motor...

Hypogonadotropic Hypogonadism

Kallmann syndrome is the most common form of isolated hypogonadotropic hypogonadism (see Chapter 5). Hypogonadism in these subjects results from GnRH deficiency. The other components of this syndrome include anosmia or hyposmia, resulting from hypoplasia of the olfactory lobes, and occasionally cleft lip and palate, unilateral renal agenesis, short metacarpals, sensory-neural hearing loss, and color blindness. Nearly 50 of Kallmann syndrome patients have mutations in the KAL gene on chromosome Xp22.3. This gene encodes an extracellular matrix protein that regulates axonal path-finding and cellular adhesion (64). Defects in this gene cause failure of fetal GnRH neurons to migrate from the olfactory palacode to the mediobasal hypothalamus, resulting in hypoplasia of the olfactory sulci. Autosomal disorders (dominant or recessive) may also cause Kallmann syndrome, but those gene defects have not yet been characterized. Because approximately half of the patients have...

Environmental Factors

Fetal Alcohol Syndrome Vulva

Cataracts, glaucoma, heart defects, deafness, teeth Microcephaly, blindness, mental retardation, fetal death Microphthalmia, microcephaly, retinal dysplasia Limb hypoplasia, mental retardation, muscle atrophy Microcephaly, growth retardation Hydrocephalus, cerebral calcifications, microphthalmia Mental retardation, deafness Caution has also been expressed regarding a number of other compounds that may damage the embryo or fetus. The most prominent among these are propylthiouracil and potassium iodide (goiter and mental retardation), streptomycin (deafness), sulfonamides (kernicterus), the antidepressant imipramine (limb deformities), tetracyclines (bone and tooth anomalies), amphetamines (oral clefts and cardiovascular abnormalities), and quinine (deafness). Finally, there is increasing evidence that aspirin (salicylates), the most commonly ingested drug during pregnancy, may harm the developing offspring when used in large doses.

Femoral neck fractures in osteogenesis imperfecta

Osteogenesis Imperfecta

Osteopetrosis a congenital and a later appearing form are known. The congenital form can be recognized by intrauterine fractures and by bone deformities already apparent at birth. Typical for this disease is the absence of periosteal bone formation. Symptoms include a transparent blue sclera, deafness, deformation of the vertebral column and foremost the extreme fragility of bone. Later appearing forms can imitate a juvenile or postmenopausal osteoporosis (Fig. 234).

Multiple Sulfatase Deficiency

Chromosomal Perivascular Spaces

The early-childhood form is the usual, or classic, form of MSD. The clinical features are those of infantile metachromatic leukodystrophy with mild signs of mucopolysaccharidosis. Early development can be normal or delayed. Affected children usually acquire the ability to stand and to say a few words, but their development is less well advanced in the presympto-matic period than that of children with infantile metachromatic leukodystrophy. During the 2nd year of life the children develop signs of a progressive en-cephalopathy with loss of acquired abilities, progressive dementia, spasticity, microcephaly, blindness, hearing loss, and difficulties in swallowing. Tendon reflexes are variable. In the final stages there is often areflexia caused by the peripheral neuropathy. Features similar to those of mucopolysaccharidosis may occur early or later in the course of the disease. These include ichthyosis, mild coarsening of the facial features, hepatosplenomegaly, stiff joints, growth...

Linked Adrenoleukodystrophy


Gait, loss of vision, and impaired auditory discrimination. The course of the disease is relentlessly progressive and spastic tetraplegia and dementia become manifest in months. Decreased vision is caused by optic atrophy or bilateral occipital white matter lesions or, more often, a combination of the two. Initially, neurological findings are often asymmetrical with hemiparesis or hemianopia. Frequently noted subsequent problems are dysarthria, dysphagia, and hearing loss. Cerebellar ataxia or sensory disturbances may be present, but are not usually prominent. There are no clinical signs of peripheral nerve dysfunction. Progressive dementia occurs. Epileptic seizures occur and are often multifocal in origin. The pace of deterioration is variable. In the final stage a spastic quadri-plegia is present and a variable degree of decorticate posturing. The affected boys are blind, deaf, and mute. A vegetative state or death is reached in 1-5 years. Most patients die within 2 or 3 years...

Cytoplasmic Inheritance

Variegated Four Clock

Are there cytoplasmic mutations in humans Some human pedigrees show transmission of a specific rare phenotype only through females and never through males. This pattern strongly suggests cytoplasmic inheritance, and would point to the phenotype being caused by a mutation in mitochondrial DNA. The disease MERRF (myoclonic epilepsy and ragged red fiber) is such a phenotype, resulting from a single base change in mitochondrial DNA. It is a muscle disease, but the symptoms also include eye and hearing disorders. Another example is Kearns-Sayre syndrome, a constellation of symptoms affecting eyes, heart, muscles and brain that is caused by loss of part of the mitochondrial DNA. In some of these cases, the cells of a sufferer contain mixtures of normal and mutant chromosomes, and the proportions of each passed on to progeny can vary as a result of cytoplas-mic segregation. The proportions in one individual can also vary in different tissues or over time. It has been proposed that the...

Diseases of the ear and mastoid process H60H95

Conductive and sensorineural hearing loss Includes congenital deafness Excludes deaf mutism NEC ( H91.3 ) deafness NOS ( H91.9 ) hearing loss H90.0 Conductive hearing loss, bilateral H90.1 Conductive hearing loss, unilateral with unrestricted hearing on the contralateral side H90.2 Conductive hearing loss, unspecified Conductive deafness NOS H90.3 Sensorineural hearing loss, bilateral H90.4 Sensorineural hearing loss, unilateral with unrestricted hearing on the contralateral side H90.5 Sensorineural hearing loss, unspecified Congenital deafness NOS Hearing loss H90.6 Mixed conductive and sensorineural hearing loss, bilateral H90.7 Mixed conductive and sensorineural hearing loss, unilateral with unrestricted hearing on the contralateral side H90.8 Mixed conductive and sensorineural hearing loss, unspecified Sensorineural deafness NOS Other hearing loss Excludes abnormal auditory perception ( H93.2 ) hearing loss as classified in H90.-impacted cerumen ( H61.2 ) noise-induced hearing...

Metabolic Bone Diseases

Radiology Signs Bone Disease

Paget disease (osteitis deformans) is a disorder of aging in which bones become overgrown and thicker, but deformed. The disease results in bowing of the long bones and distortion of the flat bones, such as those of the skull. Paget disease usually involves the bones of the axial skeleton, causing pain, fractures, and hearing loss. With time, there may be neurologic signs, heart failure, and predisposition to cancer of the bones.

Cochlear implant description

Cochlear implants are electronic device systems designed to provide functional hearing to those who have severe to profound hearing loss. The cochlear implant has both external and implanted components (see Figure 26.1). The external components include a speech processor (body-worn or ear-level), headset, and cables. The speech processor receives sound waves through a microphone and converts the acoustic signal into a radio frequency signal, which is transmitted across the skin to the implanted component of the device. The implanted component is placed under the skin in a shallow 'bed' made in the bone behind the ear. The receiver stimulator portion of the implant receives and decodes the radiofrequency signal from the speech processor and converts it to an electrical signal, which is delivered to the stimulating electrode array. The electrode array is inserted into the cochlea through a surgically drilled opening, or cochleostomy. Thus, the implant system converts sound energy into...

Prion and Fungal Diseases

In inbred lines of animals, such as purebred dogs, where breeding selection for desirable characteristics also inadvertently produces recessive inherited diseases such as hip dysplasia and deafness. Some congenital defects are considered desirably exotic in companion animals, such as curled ears or stubby tails in cats, droopy ears in rabbits, or short legs, flattened faces, or lack of hair in dogs.

Mitochondrial Encephalopathy with Lactic Acidosis and Strokelike Episodes

Hashimoto Encephalopathy Mri

Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes constitute the MELAS acronym. The disease shows maternal inheritance with considerable intrafamilial variation in expression of the disease. The age at onset varies between 3 months and 40 years, but in most cases first signs and symptoms occur before adulthood. Early development is normal in the majority of patients. The first manifestations of disease usually belong to the group of general features of encephalomyopathies. Growth disturbance and epileptic seizures are the most frequent first symptoms. The disease is progressive with increasing symptomatology. Learning disabilities, cognitive regression, exercise intolerance, and limb weakness are frequent manifestations of the disease. The myopathic features are rarely very prominent in MELAS. Stroke-like episodes are rarely early signs of the disease but have occurred before the age of 40 years in almost all patients. The stroke-like events give rise...

Cytokines In Cns Infectious Diseases 31 Bacterial Meningitis

Despite advances made in vaccination and treatment strategies, bacterial meningitis remains associated with a high mortality rate and a high incidence of neurological sequelae, particularly in very young and elderly patients. Approximately 1.2 million cases of bacterial meningitis occur annually worldwide, with 135,000 deaths (86). Long-term effects resulting from meningitis include hearing loss, hydrocephalus, and sequelae associated with parenchymal damage, including memory loss, cerebral palsy, learning disabilities, and seizures (87). The majority of community-acquired meningitis cases are caused by organisms that colonize the mucosal membranes of the nasopharynx, including Neisseria meningitidis, Streptococcus pneumoniae, and Haemophilus influenzae

Congenital Long Qt Syndrome

Congenital Lqt1

The hereditary long QT syndrome (LQTS) is a rare disorder characterized by prolongation of the QT interval on the electrocardiogram (ECG) and a propensity for syncope, torsades de pointes, ventricular arrhythmias, and sudden death. One form of LQTS, described by Jervell and Lange-Neilsen in 1957, is characterized by deafness and autosomal recessive inheritance (1). The most common form of LQTS was initially described by Romano (2) and Ward (3), and is characterized by autosomal dominant inheritance and normal hearing. Remarkable progress has been made in the last several years in our understanding of the pathogenesis of LQTS. It is now clear that LQTS is a heterogeneous disorder caused by mutations in specific ion channels that play a critical role in the control of cardiac repolarization (4). These findings have revolutionized our understanding of LQTS, and may yield new insights into other conditions characterized by ventricular arrhythmias in the absence of ischemia or structural...

Prevellance Of Tsetseflies In Mulanje District

In Malawi the mosquito is generally described as udzudzu, a term not only covering the mosquitoes (of the genera Anopheles, Aedes and Culex) but also similar flies, including crane flies (Tipulidae). People always recognize it as a troublesome pest (-bvuta kwambiri), which gives (-patsa) a person the disease (nthenda) malaria. The insecticide 'Doom' is used to rid the house of mosquitoes, if people have sufficient cash to buy it - and most rural people do not. It should be recognized, of course, that most of the mosquitoes (udzudzu) that people encounter, whether in the woodlands or urban areas, are not vectors of malaria, even though they may cause intense discomfort. It is also worth emphasizing that in the Lower Shire Valley during the rainy season - as I have experienced myself on many occasions - mosquitoes can be prodigious in their numbers, and being without a mosquito net can lead to sleepless nights. In the past people in the Lower Shire Valley slept in specially woven bags...


CMV is a frequent cause of asymptomatic infections in humans. As a result, clinical CMV disease is fairly uncommon except among neonates and immunocompromised individuals (56). Congenital CMV infection is estimated to occur among 1 of all newborns in the United States (57), making it the most common congenital infection. Among the newborns infected with CMV, approx 10 will exhibit clinical symptoms. Congenital CMV infection results in cytomegalic inclusion disease (CID) which presents as a widely disseminated infection with multiorgan involvement. CID is the leading cause of mental retardation, deafness, and other neurologic deficits among neonates (56). In immunocompromised individuals with a cell-mediated deficiency (such as bone marrow and solid organ transplant patients and individuals with AIDS), primary or reactivated CMV infections can result in several life-threatening diseases including interstitial pneumonia, gastroenteritis, hepatitis, and leukopenia (56). In addition, CMV...

F Nursing Alert

Patient taking aminoglycosides is at risk for ototoxicity. Auditory changes are irreversible, usually bilateral, and may be partial or total. The risk is greater in patients with renal impairment or those with preexisting hearing loss. It is important for the nurse to detect any problems with hearing and report them to the primary health care provider because continued administration could lead to permanent hearing loss.

Long QT Syndrome

The most common form of long QT syndrome, LQT1, which accounts for over 50 of inherited cases of the disorder, is linked to chromosome 11p15.5. It results from mutations in the K+ channel gene KCNQ1 (Wang et al, 1996). Two forms of LQT1 have been described an autosomal dominant form of the disorder, known as Romano-Ward (RW) syndrome, and a much rarer recessive form known as Jervall-Lange-Nielsen (JLN) syndrome. The major clinical difference between these syndromes is that patients with JLN syndrome suffer from profound congenital bilateral deafness, in addition to the cardiac abnormalities characteristic of all LQT disorders. Mutations in minK also give rise to LQT1 (Splawski et al., 1997). Patients who are heterozygous for the mutations D76N and S74L present with LQT syndrome, whereas homozygotes or compound heterozygotes exhibit both LQT syndrome and deafness. When coexpressed with KCNQ1, these minK mutations decrease the K+ current by shifting activation to more depolarized levels.

Clinical Syndromes

In a small proportion of persons infected with eastern, western, or Venezuelan encephalitis viruses, a few days after the onset of lever the patient develops drowsiness, often accompanied by neck rigidity, and may progress to confusion, paralysis, convulsions, and coma. Case-fatality rates average 10-20 but may be considerably higher with eastern equine encephalitis. Survivors from encephalitis caused by any of these viruses are often left with permanent neurologic sequelae such as mental retardation, epilepsy, paralysis, deafness, and blindness.

Lassa Fever

Lassa fever is very variable in its presentation, making it difficult to diagnose, whether in endemic areas or in returning travelers. It may present with insidious development of fever, headache, and malaise, progressing to a very sore throat, pains in the back, chest, and joints, vomiting, and proteinuria. In severe cases, conjunctivitis, pneumonitis, carditis, hepatitis, encephalopathy, nerve deafness, and or hemorrhages are seen, death occurring in about 20 of hospitalized cases, usually following cardiovascular collapse. Mortality is higher during the third trimester of pregnancy, and fetal loss is almost invariable.


Otitis is any inflammation of the ear. Otitis media refers to an infection that leads to the accumulation of fluid in the middle ear cavity. One cause is malfunction or obstruction of the eustachian tube, such as by allergy, enlarged adenoids, injury, or congenital abnormalities. Another cause is infection that spreads to the middle ear, most commonly from the upper respiratory tract. Continued infection may lead to accumulation of pus and perforation of the eardrum. Otitis media usually affects children under 5 years of age and may result in hearing loss. If untreated, the infection may spread to other regions of the ear and head. Treatment is with antibiotics. A tube also may be placed in the tympanic membrane to ventilate the middle ear cavity, a procedure called a myringotomy.


Encephalitis is one of the most serious of all viral diseases. The illness often begins like meningitis with fever, headache, vomiting, and neck rigidity, but alteration in the state of consciousness indicates that the brain parenchyma itself is involved. Initially lethargic, the patient becomes confused then stu-porose. Ataxia, seizures, and paralysis may develop before the victim lapses into a coma and dies. Survivors may often be left with a pathetic legacy of permanent sequelae, including mental retardation, epilepsy, paralysis, deafness, or blindness. In most of the temperate regions of the world, mumps is the commonest cause of encephalitis, but it is generally a relatively mild meningoencephalitis with only rare sequelae, mainly unilateral deafness. Herpes simplex virus is the most commonly identified cause of severe sporadic encephalitis. This is a very unpleasant disease indeed, infecting both neurons and glia to produce a focal encephalitis generally localized to the...

Refsum Disease

Refsum disease (RD), also called heredopathia atactica polyneuritiformis and hereditary sensory and motor neuropathy type IV, is a rare disorder with an autosomal recessive mode of inheritance, characterized by accumulation of phytanic acid. The age at onset of clinical signs and symptoms varies from early childhood to the fifth decade. The onset is insidious and may be difficult to determine precisely. Most patients have clear-cut manifestations before the age of 20 years. Symptoms may be precipitated by infections and low caloric intake. Dramatic exacerbations and remissions of symptoms may also occur spontaneously, without obvious antecedent cause. The main clinical features are visual disturbances, peripheral polyneuropathy, and cerebellar ataxia. Other frequently observed abnormalities are anosmia, cardiac problems, skin changes, sensorineural deafness, and skeletal abnormalities. Psychoses, particularly of a paranoid type, occur more frequently than one would expect on the basis...


Glaucoma is an abnormal increase in pressure within the eyeball. It occurs when more aqueous humor is produced than can be drained away from the eye. There is pressure on blood vessels in the eye and on the optic nerve, leading to blindness. There are many causes of glaucoma, and screening for glaucoma should be a part of every routine eye examination. Fetal infection with German measles (rubella) early in pregnancy can cause glaucoma, as well as cataracts and hearing impairment. Glaucoma is usually treated with medication to reduce pressure in the eye and occasionally is treated with surgery.

Figure 937

Vander Physiology Cochlea

Position of the stapes in the oval window. These muscles help to protect the delicate receptor apparatus of the inner ear from continuous intense sound stimuli and improve hearing over certain frequency ranges. Electronic devices can help compensate for damage to the intricate middle ear, cochlea, or neural structures. Hearing aids amplify incoming sounds, which then pass via the ear canal to the same cochlear mechanisms used by normal sound. When substantial damage has occurred, however, and hearing aids cannot correct the deafness, electronic devices known as cochlear implants may restore functional hearing. In response to sound, cochlear implants directly stimulate the cochlear nerve with tiny electric currents so that sound signals are transmitted directly to the auditory pathways, bypassing the cochlea.

Epidural Blood Patch

Once an epidural blood patch has been administered for PDPS, relief of symptoms may be almost immediate. Anecdotally, some patients may report relief of their headache even while the injection is being performed. Most patients with hearing loss secondary to CSF hypo-volemia will demonstrate significant improvement in hearing within an

Nasal Septum Cocaine

Cocaine Lesions

Wegener granulomatosis is characterized by a great variety of presentations, including systemic and local manifestations. General symptoms may be weakness, arthralgia, neurologic deficits, unexplained fever, malaise, and weight loss. Otitis media and hearing loss, subglottic stenosis, and oropharyngeal lesions, such as ulcers, are common presentations in the otolaryngologic district. However, the sinonasal tract is by far the most frequently involved area, with

GM1 Gangliosidosis

Gm1 Gangliosidosis Type

Type 1 infantile GM1 gangliosidosis presents at or soon after birth, signs being poor sucking and feeding. The child is hypotonic and hypoactive and soon develops facial and peripheral edema. At times neonatal ascites and hydrocele are seen, and sometimes generalized edema. There are characteristic coarse facial features similar to those found in mu-copolysaccharidoses. Facial abnormalities include frontal bossing,wide and depressed nasal bridge,long philtrum, and large low-set ears. The gums and tongue may appear hypertrophied. The cornea is clear. Hepatomegaly is present, and the spleen is often also enlarged. The dysmorphic features and he-patosplenomegaly gave this disease its other name pseudo-Hurler disease. The skin is usually thick and rough. Exceptional cases of angiokeratoma corporis diffusum have been described. Failure to thrive and severe psychomotor retardation are early signs of the disease. Bilateral, cherry-red spots are found on the maculae in about half the...

Hensen S Stripe

Structure Ear Facial Nerve

The process of sound transmission can bypass the ossicular chain entirely. If a vibrating object, such as a tuning fork, is placed against a bone of the skull (typically the mastoid), the vibrations are transmitted mechanically to the fluid of the inner ear, where the normal processes act to complete the hearing process. Bone conduction is used as a means of diagnosing hearing disorders that may arise because of lesions in the ossicular chain. Some hearing aids employ bone conduction to overcome such deficits.


Damage to the tympanic membrane or middle-ear ossicles produces conduction deafness. This impairment can result from a variety of causes, including otitis media and otosclerosis. In otitis media, which sometimes follows allergic reactions or respiratory disease, inflammation produces an excessive accumulation of fluid within the middle ear. This, in turn, can result in the excessive growth of epithelial tissue and damage to the eardrum. In otosclerosis, bone is resorbed and replaced by sclerotic bone that grows over the oval window and immobilizes the footplate of the stapes. In conduction deafness, these pathological changes hinder the transmission of sound waves from the air to the cochlea of the inner ear.

Kearns Sayre Syndrome

Kss Syndrome Deletion

Kearns-Sayre syndrome (KSS) is a rare, sporadic disorder that affects males and females equally. Disease onset is before the age of 20 years. The sequence of manifestations is not constant, but the signs and symptoms in themselves are consistent. Early development is normal. Ptosis and chronic progressive external ophthalmoplegia are usually the initial signs. Apart from progressive external ophthalmoplegia the typical clinical triad includes pigmentary degeneration of the retina and cardiac conduction block. The fine salt-and-pepper type of atypical retinitis pigmentosa is usually associated with good visual function and follows a benign course. Incidentally, choroi-deremia is present instead of pigmentary retinopathy. The most common cardiac conduction blocks are complete atrioventricular block, bundle branch blocks, and fascicular blocks. Other frequently noted signs are short stature due to progressive growth failure, delayed psychomotor development, sensorineur-al hearing loss,...

Vestibular Apparatus

Cupula Crista Cephalopods Scheme

Disorders of hearing are broadly divided into the categories of conductive hearing loss, related to structures of the outer and middle ear sensorineural hearing loss ( nerve deafness ), dealing with the mechanisms of the cochlea and peripheral nerves and central hearing loss, concerning processes that lie in higher portions of the central nervous system. Damage to the cochlea, especially to the hair cells of the organ of Corti, produces sensorineural hearing loss by several means. Prolonged exposure to loud occupational or recreational noises can lead to hair cell damage, including mechanical disruption of the stereocilia. Such damage is localized in the outer hair cells along the basilar membrane at a position related to the pitch of the sound that produced it. Antibiotics such as streptomycin and certain diuretics can cause rapid and irreversible damage to hair cells similar to that caused by noise, but it occurs over a broad range of frequencies. Diseases such as meningitis,...


Patients typically present with multi-focal symptoms and signs related to different levels of the neuraxis (Table 2).1,3,60 63 Frequent cerebral signs or symptoms include headache, change in mental status, nausea and vomiting, or seizures. Common spinal complaints, e.g. weakness, paresthesias in one or more extremities, back pain, radicular pain, and bladder or bowel dysfunction associated findings include asymmetries of deep tendon reflexes, nuchal rigidity or pain on straight leg raising. The most common cranial nerve complaints are diplopia, facial numbness, hearing loss, and loss of visual acuity.(3)

Diabetes Mellitus

Impaired mitochondrial metabolism can also give rise to diabetes, presumably as a consequence of a failure to regulate -cell KATP channel activity. Maternally-inherited diabetes with deafness (MIDD) results from a mutation at position 3243 of the mitochondrial DNA, which encodes a leucine transfer RNA (Maasen & Kadowaki, 1996). The disease shows maternal inheritance because all mitochondria derive from the oocyte. Confusingly, MELAS syndrome (mitochondrial myopathy, encephalopathy, lactic acidosis and strokelike episodes) is associated with the same mutation as MIDD and it is still unclear why some carriers of the 3243 mutation develop MIDD whereas others develop MELAS syndrome. The cause of deafness in MIDD is also unknown.


Iodine daily requirement derives from vegetable, rice or fish. For some people living in the most impoverished areas in high altitude or far from the sea this in not possible. Iodine once present in the soil has long since leached away, causing catastrophic effects for public health. Iodine deficiency can lead to neurological disorders, deafness, psychomotor retardation, brain damage, mental defi

Toxic Reactions

Drug toxicity can be reversible or irreversible, depending on the organs involved. Damage to the liver may be reversible because liver cells can regenerate. However, hearing loss due to damage to the eighth cranial nerve caused by toxic reaction to the anti-infective streptomycin may be permanent. Sometimes drug toxic-ity can be reversed by the administration of another drug that acts as an antidote. For example, in serious instances of digitalis toxicity, the drug Digibind may be given to counteract the effect of digoxin toxicity.

Long QT Disorders

The long QT syndromes are associated with genuinely life-threatening syncopes that may be hypotonic or convulsive. The mechanism of the syncopes is a ventricular tachyarrhythmia, normally torsades de pointes. As a rule, there is no great difficulty in the diagnosis of the syndrome of Jervell and Lange-Nielsen (37), in which congenital deafness is associated with an autosomal recessive inheritance. Much more difficult is the Romano-Ward syndrome (38), which is dominantly inherited but with incomplete penetrance. It has been suggested (39) that the diagnosis may fairly easily be made by asking the right question, in particular asking whether the child lost consciousness and remained completely still (like a dead body) for several seconds before having tonic-clonic seizures. Actually what these authors describe is a cardiogenic syncope, not fundamentally different from the reflex anoxic seizure seen with reflex asystolic syncope or a convulsive vasovagal syncope in which the vagal...


Patients can present with a variety of symptoms, which may be topographically nonspecific (vomiting, headache), focal (cranial nerve palsy, paraparesis) or multifocal (encephalopathy in conjunction with cranial nerve dysfunction).23'27 Any of these pleomorphic clinical manifestations warrant consideration of meningeal disease in patients with cancer. In general, patients present with neurologic symptoms and signs referable to three CNS domains the cerebral hemispheres, cranial nerves or spinal cord nerve roots.16 Headache, nausea and vomiting, or mental status changes suggest cerebral hemisphere involvement, whereas diplopia, facial weakness, dysphagia and hearing loss are suggestive of cranial nerve involvement. Spinal cord or nerve root involvement may cause back pain only, radiculopathy, myelopathy or paraparesis.16'23'27 Because these signs and symptoms can be vague or diffuse,

Audio Testing

Patient Ventilator Cartoon

A basic part of an otolaryngologist's practice involves diagnosing and treating patients with a hearing loss. An audiometric test booth is used for testing hearing. It is a soundproofed booth approximately 30X40 inches (or may be larger) with a door at one end. The patient sits inside and listens to sounds of different frequencies through headphones. The technician or audiologist sits outside the booth, at a counter facing the patient, and looks into the booth through a window so that the patient is always in view (Figure 4-63). This countertop contains the audio equipment from which sounds are transmitted to the patient in the booth. The patient's responses are digitally recorded, and a graph of hearing loss is produced for the physician to evaluate.

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