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!

Breaking The Sound Barriers Summary

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Author: Julie Postance
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Methods for Studying Hearing Impairment and Auditory Problems of the Aged

Age-related hearing loss presbycusis is the foremost communication disorder of our elderly, and one of their top three chronic medical conditions. Currently, there are no cures for the sensorineural hearing loss and auditory processing problems that affect the majority of persons over age 60. We hope that increased basic research with animal models coupled with human clinical studies will lead to breakthrough translational studies aimed at prevention and eventual biomedical cure. The present chapter reviews a variety of effective procedures for measuring hearing loss as a function ofage, including both classical and experimental paradigms. A theme of this exposition is that by utilizing key testing procedures, we can gain an understanding about how age and age-related ototoxic insults and conditions can affect either the cochlea (portion of the inner ear used for hearing) or the central auditory system (portions of the brain used for hearing). A classic sign and symptom of age-related...

Prevalence of hearing impairment

Hearing impairment is undoubtedly a common disease. Around 1.06 per 1000 children are born with a significant, permanent hearing impairment (40 dB or greater increase in threshold in their better hearing ear), and by the age of nine years, this number has risen to around 1.65 per 1000 (Fortnum et al., 2001). The prevalence of hearing impairment continues to increase with each decade of life, until 40 of the 71-80 years age group and 80 of the 80+ age group have a hearing loss of 35 dB or more (Davis, 1989 Davis and Moorjani, 2002). In total, approximately 20 of all adults over 18 in the UK suffer some form of hearing impairment (25 dB or greater hearing loss in at least one ear), and the proportions for other countries are very similar (Davis and Moorjani, 2002). The increase at various impairment levels is illustrated in Figure 33.1. However, thresholds are a crude reflection of the impairment, because it is not just the amplitude but the clarity of hearing that is affected. Our...

Mechanisms of hearing impairment

Figure 33.1 Prevalence of hearing loss (HL) with age, for different severities in decibels (db). Figure 33.1 Prevalence of hearing loss (HL) with age, for different severities in decibels (db). It is frequently stated that age-related hearing loss is due to degeneration of the sensory hair cells in the organ of Corti of the cochlea. However, there is no evidence for this contention from animal studies. Rather, it appears that hair cell degeneration is a correlate or a consequence of some primary dysfunction, either of hair cells or of some other part of the auditory system. Figure 33.3 Typical audiograms of 70-80 years old, sensory type. Hearing loss (HL) is shown in decibels (db). Figure 33.3 Typical audiograms of 70-80 years old, sensory type. Hearing loss (HL) is shown in decibels (db). These sensory hair cells seem to be particularly sensitive to any disturbance of their homeostasis, resulting in their degeneration. However, findings in animal models suggest that hearing...

Environmental causes of deafness

It is well established that exposure to certain environmental factors will lead to hearing loss (e.g. Fransen et al., 2003). Excessive noise exposure is the most obvious cause of hearing loss. However, infections such as prenatal rubella or meningitis can lead to deafness. Certain drugs also have ototoxic effects, including aminoglyco-side antibiotics, cisplatin and diuretic agents. In some cases it is clear that one of these factors is the immediate cause of deafness, such as hearing impairment that develops over the few days after exposure to an ototoxic drug or after meningitis, but often it is not so obvious. For example, although many studies of noise exposure in animals have shown the deleterious effects of noise on auditory function, such experiments are impossible in humans so we are left trying to piece together a history of noise exposure long after the event. The National Study of Hearing in the UK found a small but significant effect of relatively high reported noise...

Role of genetics in deafness in the population

Many reports of clinical populations of children with permanent hearing impairment have been published, usually suggesting between a third and a half of children have a genetic etiology (e.g. Fortnum et al., 2002 Morzaria et al., 2004). However, the criteria for ascribing a genetic aetiology often are not clear many authors assume a genetic causation in the absence of any other clear cause, while others report syndromic deafness as a separate category even when it is highly likely to be due to a single gene mutation. Other reports of the role of single gene mutations in hearing impairment use a selected clinical population as the denominator. Examples of this are the reports of the frequent involvement of mutations of the GJB2 gene in deafness (in some Mediterranean populations up to 50 ) the clinical populations reported often include only those with a definitive family history suggesting autosomal recessive inheritance of severe or profound deafness, excluding the sporadic cases...

Modifiers of deafness genes

Several modifier genes have been localised that modify the phenotype of carriers of known deafness genes. In humans, a dominant modifier has been reported to suppress deafness in people homozygous for the DFNB26 recessive deafness locus (Riazuddin et al., 2000). Two modifiers have been proposed to influence the hearing loss observed in carriers of the A1555G mitochondrial mutation associated with progressive hearing loss, a locus on chromosome 8 near D8S277 and a locus on chromosome 6 near the TFB1M gene (Bykhovskaya et al., 2000 2004). In the mouse, three modifiers of deafness genes have been identified. The first is moth1, which has a major effect upon the hearing of tubby mutant homozygotes (tub tub), and has been identified as a variant of the Mtap1 gene. A second is mdfw, modifier of deaf waddler, which largely determines whether heterozygous carriers of a deaf waddler mutation (+ Atp2b2dfw2J) show progressive hearing loss or not. This modifier is most likely to be a synonymous...

A model for human hearing impairment

Age-related hearing loss is an extremely common human disease, so what have we learnt so far that might suggest the causes Does the common disease common variant model look the most likely model If we look firstly at childhood deafness, despite the large number of genes that have been identified, most families with a deaf child still have no molecular diagnosis. The gene most commonly involved in severe or profound childhood deafness is GJB2, encoding the connexin 26 molecule, a component of gap junctions thought to be important for homeostatic control within the cochlear duct. Mutations in this gene account for between 25 and 50 of cases of known recessive congenital severe or profound deafness, and about 10 of sporadic cases. There are still a large number of cases with severe or less severe hearing impairment for which no gene is known. Many of the genes known to be involved in deafness affect only a handful of families. These observations suggest that childhood deafness is...

Hearing Impairments

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...

Systemic Lupus Erythematosus

Manifestations of SLE occur through a variety of pathogenetic mechanisms. Lesional skin biopsies can demonstrate inflammation and degeneration of the dermo-epidermal junction. Granular deposits of immunoglobulins and complement components can be seen, often in a band-like pattern, under immunofluorescent microscopy. Frank vasculitis can affect small blood vessels as well. Whether otorhinolaryngeal manifestations such as autoimmune hearing loss seen in association with SLE result primarily from autoantibody-mediated events, or vasculitis, or a combination of inflammatory and immune events, is largely unknown. Some neurologic manifestations of SLE have been associated with antiphospholipid antibodies, giving rise to the possibility that pathogenesis could be linked to thrombosis.

Animal Models for AIED

Animal models have definitively demonstrated that the immune response of the inner ear can lead to reversible or permanent damage to the delicate inner ear structures. One of the first animal models for AIED was developed by Beickert who immunized guinea pigs with homologous inner ear tissue. Although the guinea pigs developed cochlear lesions, hearing loss was not demonstrated and antibodies to inner ear antigens were not identified (24). Yoo developed an animal model for AIED based on type II collagen. Rats were immunized with bovine type I and type II collagen. Rats immunized with type I collagen or with denatured collagen had no change in hearing, whereas rats immunized with type II collagen had hearing loss based on auditory brainstem recordings. High levels of antibodies to type II collagen were identified. Upon sacrifice, histologic findings were of cochlear nerve degeneration and perineural vasculitis (25). Harris developed an animal model of AIED when he immunized guinea pigs...

Complicationsprognosis

The prognosis for patients with AIED is quite variable. Part of this variability can be explained by the diverse nature of the disorder. Autoimmune injury in general can be from direct antibody injury toward specific epitopes, from immune complex deposits, or from cellmediated attacks by T cells. Depending on the cause of the autoimmune process and the speed with which it is treated, the damage to the cochlea and vestibule can be reversible or permanent. Cogan's syndrome, for example, carries a poor prognosis leading to bilateral profound hearing loss in greater than 60 of cases, even with aggressive treatment (see Complications of AIED include deafness as well as oscillopsia. Cochlear implants are an excellent choice for rehabilitation of patients with AIED who have progressed to unaidable profound bilateral hearing loss. As the autoimmune process damages the inner ear, cochlear nerve function remains intact, allowing for excellent results upon cochlear implantation. There have been...

Ultrasonic vocalizations in rats

The conclusion of the devocalization study I presented in the preceding paragraph is nicely supported by a most interesting observation deafening of the male rat has the same effect as devocalization of the female. Both female darts and male pursuits are increased (White and Barfield, 1987). Here it is difficult to imagine that the female enhances her darting because she wants to make herself more attractive. It is unlikely that the females know that their partner is deaf, however exquisite their intelligence may be. Thus, the change in female behavior must be secondary to deafness-induced changes in male behavior. This proposal also

Complications And Prognosis

Rarely, patients with chronic IK may develop progressive corneal opacification, which decreases visual acuity. Such patients may require corneal transplantation. Cataracts may also occur and require extraction. Serious posterior segment eye disease can lead to decreased visual acuity. The hearing decline is the major debilitating complication of CS. Progression to deafness is frequent and occurs in approximately 25 to 50 of patients. Many patients have benefited from a cochlear implant, which has dramatically improved their quality of life. Vestibular symptoms usually improve with time, but as many as 20 of patients may have persistent oscillopsia.

Physical characteristics

Digits on fore- and hindlimbs fused to a mitten-like structure from which only the 3.2-3.9 in (8-10 cm) long claws protrude. These allow branches to be gripped without expending muscular force. The number of digits on the forelimb distinguish Bradypus from Choloepus sloths. They would be better named, three- and two-fingered sloths, since both have three digits on the hindlimbs. There are eight or nine neck vertebrae (most species of mammal, even giraffes, have seven). This allows the head to be turned with a considerable range, an important advantage for an animal with otherwise rather limited flexibility. The testes are internal. There are no incisors or canine teeth and the simple, peg-like incisors lack enamel. Sloths have poor hearing, but fairly good eyesight and smell.

Clinical Manifestations

Of the facial nerves, the ophthalmic division of the trigeminal nerve is most frequently affected in herpes zoster (Fig. 11), and this event can cause optic keratitis, a potential cause of blindness. The most visible sign of motor nerve involvement is facial paralysis as seen in Ramsay Hunt syndrome, a condition which is more properly described as herpes zoster cephalicus (Fig. 7). Patients affected with this condition also have palatal and laryngeal paralysis and hearing loss. Acute facial paralysis with pain and hearing loss is pathognomonic of herpes zoster infection. The diverse manifestations of VZV activation

Prevention of Hemophilus influenzae Type b Hib Meningitis

There are at least four reasons for preventing meningitis and other systemic infections caused by Hib (1) in the USA, Canada, Sweden, etc., Hib meningitis was common about one in 280 newborns were affected by the age of 5 years 7 . In some populations, such as Alaskan Eskimo and Australian aboriginal children, the attack rate of Hib meningitis ranged from 1 30 to 1 50 of newborns 8-10 . Patients with defective splenic function, hypogammaglobulinemia and children who live under crowded conditions and poverty are also highly susceptible (2) mortality is 5-10 and about 30 of cured patients had central nervous system (CNS) deficits ranging from deafness, seizures to mental retardation 11 (3) about 30 of isolates were ampicillin-resistant and resistance to other antibiotics

Segmental Duplications And 22q112 Rearrangement Disorders

Both intrachromosomal and interchromosomal unequal crossover events were found to be responsible for the 3-Mb deletion (Fig. 2) (26,38,39). This was determined by performing haplotype analysis with microsatellite markers, of three generations, the patient, parents, and grandparents. For interchromosomal events, which are more predominant (Baumer et al. 38 Siatta et al. 39 ), there should be individuals with the reciprocal duplication, dup(22)(q11.2q11.2). An analogous mechanism has been described for chromosome 17p11.2 for Smith-Magenis syndrome (SMS) (40). Indeed, interchromosomal unequal recombination events between two misaligned chromosomes 22 during meiosis resulted in a reciprocal duplication, dup(22)(q11.2)(11.2), (Fig. 2) (11,12,26). Unrelated dup(22)(q11.211.2) patients had been ascertained based on suspicion that they had VCFS DGS. Dup(22)(q11.2q11.2) disorder is associated with some clinical findings occurring in VCFS DGS, but also with other features which are distinct...

Head and Neck Manifestations

Tuberculous otitis media is rare and usually represents hematogenous spread. Roughly one-half of the cases have no other evidence of present or past TB. The classic clinical picture is painless otorrhea with multiple tympanic perforations, exuberant granulation tissue, early severe hearing loss, and mastoid bone necrosis (see Chapter 25 for further discussion of otorrhea). The finding of multiple tympanic membrane perforations is most likely TB, possibly pathognomonic. Nonetheless, the diagnosis is difficult, even when tissue is available. Tuberculous otitis may be complicated by facial nerve paralysis, which is discussed in detail in Chapter 29. Response to drug therapy is excellent, and surgery usually is not required.

End Organ Targets and Receptors

Children with primary IGF-1 deficiency have the same phenotype as those with GH gene deletions. A boy with severe prenatal and postnatal growth failure has been described with a homozygous partial IGF-1 gene deletion (137). His growth failure was associated with bilateral sensorineural deafness, delayed motor development, and behavioral difficulties (hyperactivity and short attention span). He did not have a significant delay in bone age or hypoglycemia. An IGFBP-3 level was normal. This case suggests that IGF-1 has a role not only in GH action, but also in CNS development and function.

Candidate Gene Studies

WFS1 gene encodes wolframin, a protein that is defective in individuals with the Wolfram syndrome. This syndrome is characterized by diabetes insipidus, juvenile diabetes, optic atrophy and deafness. Disruption of Wfs1 in mice causes overt diabetes or impaired glucose tolerance, depending on genetic background (Ishihara et al, 2004 Riggs et al, 2005). Both humans and mice deficient in Wolframin show pancreatic P-cell loss, possibly as a result of an enhanced endoplasmic reticulum stress response leading to increased P-cell apoptosis (Riggs et al, 2005 Yamada et al, 2006). Hence, WFS1 is critical for survival and function of insulin-producing pancreatic beta cells. The first evidence that variation in the WFS1 gene influences susceptibility to type 2 diabetes was shown in a family-based association study (Minton et al, 2002). A study on 1,536 SNPs in 84 candidate genes using a gene-centric approach showed that only WFS1 gene was associated with type 2 diabetes (Sandhu et al, 2007)....

Radiation Oncology Nurse

Radiation therapy provides an important role in the management of HGG. Nursing care provided by the radiation oncology nurse begins at the initial consult visit and continues into the early post-radiation phase. Patients and families must be educated regarding treatment schedules and expected effects and side effects. Options available to patients include conventional regional radiation, whole brain radiation, stereotactic radiosurgery such as gamma knife or photon beam, brachytherapy, and hyperthermia (31). It is often difficult to assess if symptoms are being caused by the radiation therapy itself, tumor growth, or other concurrent therapies. Acute symptoms occur within the first few weeks to months and are usually self-limiting. These include nausea but rarely vomiting, anorexia, impaired taste, fatigue, increased seizures, increased neurologic deficits, skin changes, hair loss, and impaired wound healing. Some patients whose radiation fields lie near the ear can experience hearing...

Rocky Mountain Spotted Fever Rmsf

RMSF is an acute febrile systemic tick illness with significant mortality caused by the organism Rickettsia rickettsii. The infection is most commonly seen in the southeast United States. The incubation period is between 2 and 14 days. The symptoms are nonspecific, with fever, severe headache, myalgias, prostration, and nausea vomiting. The characteristic maculopapular rash usually appears on the fourth febrile day on the wrists, ankles, palms, soles, and forearms. A vasculitis may develop that can cause hypotension, edema, and petechiae. Specific organ involvement includes cardiac (car-diomegaly, myocarditis, EKG changes), pulmonary involvement (interstitial pneumonitis), and neurologic (mild headache to lethargy, seizures, coma, transient deafness, tremor, rigidity, paralysis, ataxia, aphasia, blindness). The initial diagnosis is often made clinically. Confirmation is obtained by serology, skin biopsy, or direct isolation and identification of the organism. Treatment is with...

Jugular foramen syndrome

Primary lesions of the jugular foramen include glomus jugulare tumors, schwannomas, and meningiomas. Metastatic lesions to the jugular foramen are more common than primary neoplasms (36,38-42). Glomus jugulare tumors are the most common primary neoplasms of the jugular foramen. These are vascular tumors arising from neuroectodermally derived paraganglia present at the jugular fossa, the inferior tympanic canaliculus, the promontory, and within the vagus nerve. Paraganglia are very similar histologically and embryologically to the adrenal medulla. They, along with their derivative glomus tumors (jugulare and tympanicum), are typically supplied by the inferior tympanic branch of the ascending pharyngeal artery (43) however, the blood supply to glomus tumors may be very extensive, originating from the external carotid, the internal carotid, and the vertebral arteries (44). These tumors are more common in females (6 1 female-to-male ratio) and usually present in middle age. Functional...

SdyDBA and sdyBL6 mice

As noted above, the original sdy mice were Dtnbpl mutants of the DBA 2J strain. This strain, unlike C57BL 6 mice, is homozygous for six mutations related to neurological, melanogenic, and or inflammatory disorders as documented by Cox et al. (2009) and Talbot et al. (2009). These include cadherin 23ahl (Cdh23ahl Cdh753A) associated with an age-related hearing loss, as well as glycoprotein (transmembrane) nmbR150X (GpnmbR150X) and tyrosinase-related protein 1isa (Tyrp1isa) associated with pigmentary glaucoma. These mutations must be present in sdy mice derived from DBA 2J animals (i.e., sdy DBA mice), though not all such mice are necessarily homozygous for them since some have wild-type Gpnmb (Anderson et al., 2002). These various mutations in DBA 2J mice appear to account for several developmental abnormalities in such animals compared to C57BL 6 mice as discussed elsewhere (Talbot et al., 2009). DBA 2J mice develop a high-frequency hearing loss between 3 and 4 weeks of age. By 5...

Lessons from other monogenic syndromes

Tissues and systems) which can result in T2D. One of the most instructive of these syndromes (although not strictly a monogenic condition) is that associated with a mutation at position 3243 in the mitochondrial genome, within the gene encoding the non-nuclear form of tRNA (leucine). Depending on the tissue load of the abnormal mitochondrial sequence in any given individual, this mutation can result in either MELAS (a neurological syndrome characterized by Myalgic Encephalopathy, Lactic Acidosis and Stroke-like episodes) or Maternally-Inherited Diabetes and Deafness (MIDD) (Kadowaki et al., 1994). The molecular mechanism here is thought to be one of disrupted mitochondrial metabolism, resulting in reduced oxidative phosphorylation, and ATP depletion. In the beta-cell this compromises glucose-stimulated insulin secretion.

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.

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...

Methodological Issues

In reviewing the preceding literature, a number of methodological procedures suggest themselves for future research on aging and auditory scene analysis. First, and somewhat obvious, it is essential that participants be screened for hearing impairment in both ears. In particular, the integrity of the cochlea should be assessed in a more comprehensive way than just measuring pure tone thresholds in an effort to dissociate nonthreshold-changing peripheral deficits from true central deficits in auditory processing. One test that could provide some information is a distortion-product otoacoustic emission (OAE). Although outer hair cells must be functioning to some degree in order to measure normal thresholds, OAEs are a more sensitive measure of outer hair cell health than pure-tone audiometry and may prove useful in assessing cochlear damage in general. Another potentially useful test would be fine-scale audiometry, which consists of obtaining thresholds with a greater degree of...

Wegeners Granulomatosis

Wegener's granulomatosis is an idiopathic vasculitis of small arteries, arterioles, and capillaries primarily affecting the upper aerodigestive tract, lungs, and kidneys. Typical presentation is in middle-aged patients with a slight male predominance. Otologic manifestations are common and range from 19 to 45 of cases (103). They include conductive hearing loss, sensorineural hearing loss, otalgia, otorrhea, and serous otitis media. Neurologic involvement is frequent with nearly half of patients demonstrating either peripheral or central neuropathy (104). Cranial nerve involvement was reported in 6.5 of patients (105). When the ear is affected by the disorder, facial nerve involvement has been estimated at 5 (103,106). Injury to the facial nerve may be from destructive granulomatous lesions involving the skull base, necrotizing vasculitis, or compressive effect due to granuloma in the middle ear (107). Pathologically, the disorder is characterized by noncaseating granulomas with...

Congenital HCMV Infection A Major Public Health Problem

The problem of congenital HCMV infection is unquestionably the major driving force behind efforts to develop a HCMV vaccine. In the developed world, HCMV is the most common congenital viral infection (Whitley 1994). Estimates of the prevalence of congenital HCMV infection suggest that between 0.5 and 2 of all newborns in the developed world are infected in utero (Demmler 1996). In the United States alone, this corresponds to approximately 40,000 infected newborn infants born annually with HCMV infection. The concern is particularly acute for HCMV-seronegative women of child-bearing age. Based on recent HCMV incidence estimates, approximately 27,000 new infections are believed to occur among seronegative pregnant women in the United States each year (Colugnati et al. 2007). Approximately 10 of congenitally infected infants have clinically evident disease in the newborn period, including visceral organomegaly, microcephaly with intracranial calcifications, chorioretinitis, and skin...

Pagets disease of bone

Paget's disease of bone (PDB) is one of the most common chronic skeletal diseases affecting up to 3 of many White populations over the age of 60 years. Its ethnic and geographic distribution is variable, with a high prevalence in Whites from the United Kingdom, Australia, North America and Western Europe. PDB is characterized by focal areas of increased bone resorption and formation, leading to deformity and or enlargement. The axial skeleton (pelvis, lumbar and thoracic spine, and sacrum) is most frequently involved, followed by the femur, skull, and tibia. The newly formed bone in pagetic lesions is disorganized, frequently resulting in bowing and increased fracture. Bony overgrowth in the skull may lead to nerve entrapment, headache, and deafness.

Familial expansile osteolysis

Familial expansile osteolysis (FEO) is characterized by focal regions of increased bone remodeling resembling those seen in adult PDB. There is increased activity of osteoclasts and osteoblasts leading to expansion, deformity and increased risk of fracture. Although PDB and FEO have histologic and phenotypic similarities, they are sufficiently different to be considered separate diseases. FEO begins in the second decade, tends to be more severe, and the bone lesions are predominantly peripheral. Pain is prominent with increased medullary expansion leading to deformity and increased risk of fracture. Many affected individuals have early onset deafness and loss of dentition. FEO has been mapped to the TNFRSF11A gene which encodes RANK, where two different tandem duplications in exon 1 encoding the signal peptide have beeen found in several families (Hughes et al., 2000). Signal peptide cleavage is defective, leading to intracel-lular accumulation of RANK and increased

Van Buchems disease and sclerosteosis

Van Buchem's disease and sclerosteosis have very similar phenotypes with large increases in the amount of bone tissue (Janssens and Van Hul, 2002). Van Buchem's disease usually begins during puberty, with osteosclerosis of the skull, mandible, clavicles, ribs, and long bone diaphyses. In scle-rosteosis mainly the skull and mandible are affected, although osteosclerosis and hyperostosis can be more generalized. In both disorders optic atrophy and deafness can occur secondary to bone encroachment on nerves. Facial paralysis is common in sclerosteosis, and may be present at birth or develop shortly thereafter. Sclerosteosis tends to be more severe, and excess height and weight (gigantism) and syndactyly are found in most patients. In a cohort of South Africans followed over a 38-year period, almost half died during the course of the survey (mean age of 33 years), usually from increased intracranial pressure (Hamersma et al., 2003). The gene(s) responsible for van Buchem's disease and...

Craniometaphyseal dysplasia

Craniometaphyseal dysplasia (CMD) is characterized by metaphyseal dysplasia with sclerosis and hyperostosis of cranial bones. CMD is usually autosomal dominant, although autosomal recessive inheritance has been reported. The long bones have broadened metaphyses and develop an ''Erlenmeyer flask'' appearance. Hyperostosis of calvarial and facial bones leads to nasal obstruction, compression of the cranial nerves, and hypertelorism. Much of the disability associated with CMD is due to facial nerve palsy and hearing loss secondary to nerve compression. Linkage between autosomal dominant CMD and chromosome 5p15.2-p14.1 has been established in three kindreds (Chandler et al., 2001 Nurnberg et al., 1997). Studies of the positional candidate gene, ANKH, have revealed several missense mutations, deletions and insertions (Nurnberg et al., 2001 Reichenberger et al., 2001). ANKH encodes a transmembrane protein (ANKH) expressed in osteoblasts that transports inorganic pyrophosphate (PPi) (Figure...

Physical Psychological And Socioeconomic Sequelae

The main difficulties faced by former child soldiers and those trying to help them are limited healthcare access and social stigma. Local hospitals do not have the equipment or the medications to provide adequate treatment, and as a result many child soldiers go without medical attention for long periods of time. Common combat-related injuries include hearing loss, blindness, and limb amputations, usually from landmines or grenades. Prostheses are in high demand because so many amputees are still growing and need frequent replacements. Sexually transmitted diseases (STDs), malaria, skin and respiratory diseases, malnutrition, bone deformation from carrying heavy loads, as well as drug and alcohol addiction are widespread.

Specific language impairment SLI

SLI is estimated to affect 2-7 of pre-primary school children and is diagnosed if significant language deficits are present despite adequate educational opportunity and normal nonverbal intelligence (Law etal., 1998). Alternative diagnoses of medical and developmental disorders such as deafness and autism must be excluded before a diagnosis of SLI is given. Features such as the orofacial dyspraxia found in the KE family would exclude a diagnosis of SLI. A continuum of language impairment exists, depending on the level of articulation and verbal expression, the ability to produce speech sounds, and verbal comprehension of others. SLI has been classified into subtypes, but it is possible that the variability seen in the phenotype reflects severity of impairment and the developmental stage of the child rather than an expression of specific deficits. Early evidence from a longitudinal study of language-impaired twins found that the majority (65.9 ) of individuals diagnosed with early...

Cochlear Physiological Potentials

Willott and coworkers were the first to systematically investigate changes in tonotopic or cochleotopic organizations of brainstem auditory nuclei as the high-frequency hearing loss characteristic of presbycusis progresses in mice (Willott et al., 1985, 1988a, b Willott, 1986, 1991). Tonotopic or cochleotopic organization refers to the fact that the major cell groups, or nuclei, of the brainstem auditory system have frequency organizations that originate in the cochlear spiral, where high frequency nerve cells are in one location, then middle frequency, then low frequency nerve cells in another, much like the keys on a piano. Tonotopically organized nerve cell pathways connect the cochlea to the cochlear nucleus, the first major nucleus of the central auditory system, and these pathways continue in an organized manner up through the auditory cortex, with sound information being processed at each step along the way. In aging, when the basal portions of the cochlea lose a significant...

With Many Different Genetic Abnormalities Conclusion From Animal Models

Congenital neuromuscular disorders of the gut are commonly encountered during the neonatal period. These conditions include, in addition to Hirschsprung's disease (long and short segment varieties), the allied disorders, hypoganglionosis, neuronal intestinal dysplasias (hyper-ganglionosis), ganglion cell immaturity, and dysgangli-onoses. There are also additional defects such as hyper-trophic pyloric stenosis, volvulus, and intussusception, that may also involve abnormalities of the development of the ENS. Hirschsprung's disease is quite common and occurs in up to 1 in 5,000 live births 267 . In some patients, Hirschsprung's disease has been shown to be associated with loss-of-function mutations in the RET pro-tooncogene 267-271 . Only a small minority of patients with Hirschsprung's disease can be accounted for by RET mutations 267-269 . Both long and short segment Hirschsprung's disease can occur in patients with identical Ret abnormalities and patients may also exhibit other...

Genes and environment

Some cases of hearing impairment result primarily from a single gene mutation inherited in a Mendelian fashion, and other cases are the consequence of a known environmental insult such as meningitis or cisplatin administration. However, in many (maybe most) cases, both the genome of an individual and the environment interact to lead to deafness. For example, not all cases of meningitis lead to hearing loss, and people working in the same noisy environment do not all develop the same degree of hearing impairment, and the genetic susceptibility of the individuals probably accounts for these differences. We already know one mutation, A1555G in the mitochondrial genome, which makes carriers especially sensitive to aminoglycoside-induced hearing loss. This same mutation is associated with age-related progressive hearing loss in some families, irrespective of exposure to any drug. Gene variants that make carriers more susceptible to noise-induced deafness are difficult to find in humans,...

Secondary Malignancies

Audiometry assessment of a patient after cisplatin-based chemotherapy, demonstrating bilateral hearing impairment, most notably at higher frequencies. O right ear X left ear. Figure 28-5. Audiometry assessment of a patient after cisplatin-based chemotherapy, demonstrating bilateral hearing impairment, most notably at higher frequencies. O right ear X left ear.

Genetic heterogeneity

It is clear that deafness is an extremely heterogeneous disease. There are likely to be hundreds of different genes involved, any one of which can underlie deafness. For syndromic deafness, there are over 400 distinct Mendelian disorders that include deafness as one of the features listed in Online Mendelian Inheritance in Man (OMIM). For non-syndromic deafness, over 100 loci have been found and 36 of the genes have been identified (Van Camp and Smith, 2005 Petit et al., 2001 Bitner-Glindzicz, 2002 Friedman and Griffith, 2003). The genes represent a wide variety of molecules, ranging from myosin motors to transcription factors, ion channels to extracellular matrix components. They are expressed in diverse cell types within the auditory system, but mostly within the inner ear (Van Camp and Smith, 2005, see link to expression). As the genes involved in non-syndromic deafness have been localized, an interesting feature has emerged recessive deafness is mostly early-onset childhood...

Healthcare Costs Associated with Congenital HCMV Infection A Compelling Argument for Vaccine Development

The economic burden on the healthcare system in caring for neurodevelopmental disability in early childhood caused by congenital HCMV infection is substantial. Congenital HCMV infection is the most common infectious cause of brain damage in children, and HCMV causes more hearing loss in children than did Haemophilus influenzae meningitis in the pre-Hib vaccine era (Pass 1996). The economic costs to society associated with congenital HCMV infection present a compelling argument for vaccine development. In the early 1990s, the expense to the US healthcare system associated with congenital HCMV infection was estimated at approximately 1.9 billion annually, with an average cost per child of over 300,000 (Aran et al. 2004). Children with congenital HCMV infection often require long-term custodial care and extensive medical and surgical interventions. A recent economic analysis by the Institute of Medicine (IOM) examined the theoretical cost-effectiveness of a hypothetical HCMV vaccine...

Neck Vsh Investigation

Temporal bone and middle ear tumors often present with unilateral otorrhea and conductive hearing loss. As the tumor progresses, facial nerve paralysis and other cranial nerve neuropathies occur. Other head and neck manifestations include nasal obstruction, facial swelling, oropharyngeal mass, and unilateral proptosis. Metastatic rhabdomyosar-coma is part of the differential diagnosis for pediatric neck masses (23).

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.

Scope Of Ethical Issues Addressed In This Chapter

G, a 79-year-old widowed, white woman, has been admitted to the short-stay unit of a comprehensive geriatric health care center after discharge from the hospital for hip replacement. She has been discharged to the short-stay unit as part of a contract arrangement between the hospital and the geriatric health care center. Her care plan includes postsurgical follow-up, physical therapy, and planning for the future. Her medical problems include mild heart failure and moderate visual impairment, secondary to macular degeneration. She also has moderate right-side hearing loss. Her poor eyesight contributed to the fall that resulted in her broken hip. This fall occurred at home, where Mrs. G lives alone. She has lived in this home for 50 years and alone in this home since her husband died 10 years ago. She has three children, her oldest, a son, and two daughters, all of whom are married with children of their own. She is closest to her youngest child, who is 49 and has three teenage...

Scleroderma

Sensorineural hearing loss has been reported to occur, but has not been found to correlate with other disease manifestations (20,21). Virtually the entire gastrointestinal tract can be affected in scleroderma. Swallowing dysfunction due to oropharyngeal involvement can occur (22) and increase the risk of aspiration. Esophageal dysmotility and nonobstructive dysphagia are seen in the majority of patients, making symptoms of gastroesophageal reflux very common.

Case 2

A 43-year-old woman was reported who was referred for evaluation because of minor facial anomalies, myopathy, sterility, short stature, hearing loss, downward slant of palpebral fissures, bilateral ptosis, severe micro retrognathia, high arched palate, and scoliosis (Chen et al., 1999). Cytogenetic analyses showed the presence of one i(1p) and one i(1q) without normal chromosome 1 homologues. Fluorescence in situ hybridization analysis showed hybridization to only two chromosomes, consistent with the G-banded interpretation of i(1p) and i(1q). Molecular investigations using markers for chromosome 1 showed inheritance of only one set of paternal alleles and absence of any maternal alleles in the patient. The authors concluded that the adverse phenotype of the patient may be due to

Summary

Systemic rheumatic disease may present with a variety of manifestations in the head and neck regions. Especially important among these are the dermatologic findings of SLE, DM, and scleroderma. Rashes characteristic of these disorders may also arise later in the course of the diseases. Some of the more frequently reported manifestations to be aware of are autoimmune hearing loss, especially in SLE esophageal dysmotility in scleroderma oropharyngeal and esophageal involvement in DM PM and keratoconjunctivitis sicca and cervical spine involvement in RA.

Policy Matters

Some individuals worry, for example, that ''artificially'' extending human lives would cheapen our existence, whereas others point out that the modern medical enterprise has already drastically increased lifespans with no ill effects on society. Other subjects that the site has explored include the paucity of older people included in clinical trials the wisdom of using human growth hormone to combat symptoms of aging despite data suggesting that the substance curtails lifespan and how we might improve our flu-combating measures. It has discussed nanotech-nology, hormone replacement therapy, guidelines for keeping bones strong, and chronic pain, as well as age-related voice changes and hearing loss. SAGE Crossroads ponders such topics in News and Views articles as well as through Webcasts in which experts debate and discuss such matters.

Definition

AIED can be defined as a fluctuating or rapidly progressing sensorineural hearing loss that is responsive to immunosuppressive therapy. Vestibular dysfunction may or may not be present. In some cases, a concurrent systemic autoimmune disorder exists at the time of diagnosis. Therefore, AIED can exist in isolation as an organ-specific disease (primary AIED) or can occur as a nonspecific injury as part of a systemic autoimmune disease (secondary AIED). Currently, there is no diagnostic laboratory test or imaging modality available to confirm the diagnosis with certainty.

Ginkgo biloba

Controlled studies suggest that administration of Ginkgo biloba (GB) extract has limited effectiveness in improving memory and cognition, either in elderly subjects with dementia or healthy subjects. GB administration does seem to reverse sudden hearing loss in patients with mild cases of this disorder. Additionally, GB administration may blunt the rise in blood pressure in response to stress and may blunt the glycemic response after an oral glucose tolerance test. Despite the lack of evidence of effects on coagulation in vivo, a number of case reports of excessive bleeding in patients taking GB have been reported. Finally, GB does not appear to be prone to causing drug interactions, except for agents metabolized by cytochrome P450 2C19 (in which case, induction is observed).

Clinical Features

Lassa Fever does not have a pronounced hemorrhagic component (17 ), and neurological impairment is rare except for sensorineural deafness (the most common cause of deafness in West Africa). On the other hand, Bolivian Hemorrhagic Fever has neurological impairment as a prominent feature (headache, tremor, encephalitis).

Complications

About one in eight patients with herpes zoster infection has at least one complication of this condition. Major complications include postherpetic neuralgia, uveitis, motor deficits, skin infection, and systemic involvement (with manifestations such as meningoencephalitis, pneumonia, deafness, or dissemination). Postherpetic neuralgia occurs most frequently in patients older than 50 years of age and can be prolonged and intractable despite early antiviral therapy. The pain is often excruciating and does not respond well to conventional methods of pain control. Granulomatous vasculitis has recently been added to the list of complications (25).

Delusional Disorders

Delusions sometimes form part of the symptom picture in schizophrenia, particularly in younger patients, but delusions without schizophrenia are more common. The frequency of delusional disorders tends to increase with age, being exceeded only by dementia and depression in older adults. The delusions of older patients are often accompanied by a chronic hearing loss, which makes it difficult for them to understand what other people are saying and invites suspiciousness, and by cognitive disturbances, as in Alzheimer's disease. Older loners who have lived most of their lives in relative isolation from other people are more likely to be delusional than individuals with normal social interactions (Berger & Zarit, 1978).

Cochlea

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.

Cochlear Nerve

Lesions of this special sensory nerve cause deafness. Nerve cells are in the spiral ganglion of the cochlea. Their peripheral connections are to the auditory cells of the organ of Corti, and their central connections are to the cochlear nucleus. The next-order neuron is the lateral lemniscus. When impulses concerned with sound have reached the cochlear nucleus, their further passage is bilateral (ie, a unilateral lesion above the level of the nucleus cannot cause deafness). Deafness Does the patient have some hearing loss, and if so, is it nerve deafness (or sensorineural deafness) or conductive deafness Conductive deafness is caused by a disease of the external canal, middle ear, or ossicles. The key features of nerve deafness are If the patient is deaf in one or both ears, do the following to establish whether the deafness is caused by a defect in the conducting system of the ear or in the nerve leading from the ear. If the sound reappears, one can say that air conduction is better...

Coloring

One of the first signs of domestication is variability of color. The individuals all have white spots or all white or all black. It is interesting that white coloring is usually connected with lower performance (there are few white racing horses and even fewer winners) or with different defects (white noble cats have a high incidence of deafness).

Clinical Overview

Physical manifestations of WBS usually include involvement of the cardiovascular system, most often as a narrowing of the ascending aorta (SVAS) although a generalized arteriopathy can lead to vascular stenoses in other vessels, and hypertension is common in later life. Stellate irides, flat nasal bridge, short, up-turned nose with anteverted nostrils, long philtrum, full lips and lower cheeks, and a small chin are the recognizable facial features. Other symptoms include hernias, visual impairment, hypersensitivity to sound, chronic otitis media, malocclusion, small or missing teeth, renal anomalies, constipation, vomiting, growth deficiency, infantile hypercalcemia, musculoskeletal abnormalities, and a hoarse voice (11,12). As WBS individuals grow older they may also present with premature graying of the hair, diabetes and impaired glucose tolerance, decreased bone mineral density, sensorineural hearing loss, and a high frequency of psychiatric symptoms (13).

Congenital Syphilis

Children who survive the early disease manifestations will then enter a latent phase. Late symptoms may present years to decades later. Skeletal defects from the osteo-chondritis and periostitis are quite notable, with characteristic facies consisting of a high arched palate, protruding mandible, frontal bossing, and saddle-nose deformity. Other structural defects include palatal and nasal septum perforations, anterior bowing of the lower extremities known as saber shins, and bilateral knee effusions termed Clutton's joints. Hutchinson's triad consists of eighth nerve deafness, interstitial keratitis, and peg-shaped, centrally notched, widely spaced central incisors known as Hutchinson's teeth. Congenital otosyphilis is usually more severe than that seen with acquired syphilis. It typically presents with sudden onset, profound, bilateral hearing loss with rare vestibular symptoms. Untreated, congenital syphilis frequently progresses to neurosyphilis with sequelae similar to the...

Fibrous dysplasia

Temporal bone involvement is uncommon. The most common presentations are hearing loss (80 ), mass (26-50 ), draining ear (14-30 ), otalgia (7-10 ), and trismus (2.3-10 ) (1). A conductive hearing loss or canal cholesteatoma may result from collapse of the external auditory canal, if it is extensively involved. The otic capsule and or internal auditory canal are rarely involved. This involvement is associated with sensorineural hearing loss, which may be profound (5). Facial nerve involvement is not unusual (5-10 ).

Inv Dup22

A proportion of CES chromosomes have one or two copies of the DGS VCFS region in additional to four copies of the CES region. Duplications of the DGS VCFS region have been associated recently with variable symptoms including learning disabilities, cognitive and behavioral abnormalities, palate defects, hearing loss, heart defects, growth deficiency, developmental and motor delays, and urogenital anomalies (56). This likely represents only a spectrum of the microduplication 22q11.2 syndrome, because the patients were ascertained during testing specific for the DGS VCFS deletion. In any case, one would assume that the addition of extra copies of the DGS VCFS region would worsen the phenotype of CES, but this has not been observed. However, because only 10 CES patients have been characterized in this way (9), this lack of correlation is likely to be because of the extreme variability of the overall syndrome masking more subtle changes brought on by additional copies of the DGS VCFS

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.

Genetic Diseases

Congenital hearing loss is common, and about one-half of cases are thought to be genetic. Genetic hearing loss is typically classified as syndromic or nonsyndromic. When hearing loss consistently coexists with other pathologic findings, it is considered part of a syndrome when it exists in isolation, it is nonsyndromic. As our focus is on systemic diseases, nonsyndromic hearing loss, which accounts for two-thirds of hereditary hearing loss, will not be discussed extensively. Mutations in the GJB2 gene coding for connexin 26 are now thought to account for over half of the cases of nonsyndromic genetic hearing loss (1). Nearly 100 mutations have been identified, some being quite specific to individual ethnic groups. Hearing loss tends to be moderate to severe, symmetric, and nonprogressive. There are no associated temporal bone malformations, and patients tend to do well after cochlear implantation. There are more than 400 syndromes associated with sensorineural hearing loss. It is...

Medications

Many treatments have been advocated for sudden sensorineural hearing loss, which is presumed to be of viral (or rarely vascular) origin. The diagnosis of autoimmune inner-ear disease is still predicated by a documented response to corticosteroid administration (20). Treatment strategies have varied between reports, but, in general, a dose of prednisone, 40 to 60 mg in the adult for two weeks, is an appropriate therapeutic trial. Substantial improvement on this dosage regimen would then warrant a slow taper of steroids over several months with close observation for recurrence. Even prompt administration of these There are several circumstances that warrant special mention. Patients with M ni re's disease and certain forms of genetic hearing loss may manifest spontaneous fluctuations in hearing. In these situations, the administration of steroids at the onset of the hearing loss may result in a false impression that the subsequent spontaneous improvement in hearing was actually the...

Prosthetics

Hearing-aid amplification remains the mainstay of rehabilitation for patients with sensorineural hearing loss. Unfortunately, hearings aids are amplifiers, not clarifiers, and, as such, leave patients less than satisfied. Cochlear implants have revolutionized the management of patients with severe sensorineural hearing loss or deafness. They are the only prosthesis that successfully replaces sensory function. Many objective studies have proven their value in the treatment of pediatric patients' prelingual and postlingual severe-to-profound sensorineural hearing loss, and in adults with postlingual sensorineural hearing loss. Implant devices and surgical techniques have been refined since their introduction and surgical complications are rare. This remains a rapidly expanding and exciting field as software and hardware become more sophisticated and indications for use of these devices broaden.

Tuberculosis

The treatment of facial palsy in active tubercular otitis media or mastoiditis is controversial. Singh published a review of 43 patients to assess the role of surgery in tuberculous mastoiditis. Of the patients, 17 had facial paralysis in this study. He found that 92 of patients treated with antituberculosis medical therapy without surgery had recovery of the facial nerve, with only 80 of patients undergoing surgery (cortical mastoidectomy and removal of sequestrum) obtaining recovery. In the surgical patients, facial nerve decompression was not performed as a part of the operation. These results may reflect more severe cases being selected into the surgical group. The authors concluded that the only role for surgery is incision and drainage of a postauricular abscess or removal of sequestrum, if present and chemotherapeutic management is the treatment of choice for tubercular mastoiditis (22). Harbert in 1964 had a similar conclusion and felt that surgical intervention was...

Osteopetroses

One subtype of osteopetroses, termed sclerosteosis, has a proclivity for facial nerve involvement. It is characterized by syndactyly, conductive hearing loss, and sclerosis, and almost always has progressive recurrent facial nerve palsy (123). Attacks of facial paralysis commonly begin in childhood, are recurrent, and often alternate sides of the face. Total nerve degeneration is the rule and recovery with significant synkinesis at three to five months is the usual outcome (124).

Cytomegalovirus

CMV is a double-stranded DNA virus that, in normal individuals, induces a febrile illness that resembles mononucleosis from EBV, with chills, fatigue, headache, and malaise (Gandhi and Khanna 2004). In immunocompromised patients, such as those who have undergone bone marrow or other organ transplantation or who have advanced human immunodeficiency virus (HIV) disease, CMV can cause considerable morbidity and mortality. CMV may be reactivated in a previously infected person who becomes immunosuppressed. Alternatively, a de novo CMV infection may be transmitted to a CMV-negative recipient of an organ from a CMV-positive donor. Many organs can be affected by CMV infection, including the retina, lung, liver, esophagus, or colon. CMV can also cause complications to a fetus infected in utero, including hearing loss, visual loss, and neurological complications (Fowler et al. 1992). CMV hyperimmune globulins (CMVIG) first demonstrated efficacy in the treatment of disease associated with...

Therapy Evaluation

The first component of both the PT and the OT evaluation is to take the history of a PD patient. It is important to determine at what state in the disease process the patient is presenting to the therapist. Information is obtained regarding the patient's ability to perform a variety of functional tasks, including bed mobility, transfers, community ambulation, activities of daily living, work tasks, and recreational tasks. It is important to determine the patient's medication schedule and any on off fluctuations that may affect exercise performance. An account of freezing episodes, falls or near falls, or any specific situations in which mobility seems to be compromised needs to be noted. Comorbidities such as high blood pressure, heart disease, diabetes, pulmonary problems, cancer history, recent surgical history, depression, and dementia are all factors that affect the patient's ability to participate in a rehabilitation program and must be assessed. It is also important to obtain a...

Treatments

There are no treatments available, only two main types of prosthesis. First is the hearing aid, which amplifies incoming sounds and can be adjusted to amplify certain frequencies more than others to match the pattern of hearing loss of an individual, and to match the limited dynamic range of the damaged ear to avoid painfully loud sounds being delivered. However, there is much to be learnt about how we use the temporal and frequency cues in speech and other sounds in order to improve programming of hearing aids to facilitate use of these cues. The threshold for provision of a hearing aid is often considered to be around 25 dB hearing level, although many people are not fitted with an aid until their hearing is much worse than this (Davis and Moorjani, 2002). Second is the cochlear implant, which involves surgery to place an extended array of electrodes within the cochlear duct and a subcutaneous receiver for detecting the coded stimulation and transmitting to the electrode array. This...

Acknowledgements

Supported by the Wellcome Trust, MRC, Defeating Deafness and EC. Bitner-Glindzicz, M. (2002). Hereditary deafness and phenotyping in humans. Br Med Bull., 63, 73-94. Bykhovskaya, Y. et al. (2000). Candidate locus for a nuclear modifier gene for maternally inherited deafness. Am J Hum Genet., 66, 1905-10. Bykhovskaya, Y. et al. (2004). Human mitochondrial transcription factor B1 as a modifier gene for hearing loss associated with the mitochondrial A1555G mutation. Mol Genet Metab., 82, 27-32. Coling, D.E. etal. (2003). Effect of SOD1 overexpression on age- and noise-related hearing loss. Free Radic Biol Med., 34, 873-80. Davis, A. C. (1989). The prevalence of hearing impairment and reported hearing disability among adults in Great Britain. Int J Epidemiol., 18, 911-17. Fechter, L. D. (2004). Promotion of noise-induced hearing loss by chemical contaminants. J Toxicol Environ Health A., 67, 727-40. Fortnum, H. M. et al. (2001). Prevalence of permanent childhood hearing impairment in the...

Neurotoxicity

The ototoxicity associated with cisplatin is high-frequency hearing loss and is related to the cumulative dose and rate of infusion.32,33 It occurs in 20 to 40 of patients.15 Older age, serum creatinine level > 1.5 mg dL, cumulative doses of cisplatin, and preexisting hearing impairment are predictive for oto-toxicity (Figure 28-5). Although there may be some improvement over months to years, recovery to normal hearing is rare if a clinical or symptomatic hearing deficit is noted by a patient. When higher single doses of cisplatin are used, up to 60 of patients may exhibit occult audiometric changes.14

Infectious Diseases

Hearing loss is an uncommon presenting symptom of a systemic infectious disease but may develop during the course of several viral and bacterial infections. Pathogens may infect the inner ear and auditory pathways in utero, resulting in congenital syndromes that often include deafness, or infection may be acquired after birth. As the fluids of the inner ear are not easily accessible for sampling, the implication of viral pathogens as the causes of sensorineural hearing loss has often depended on circumstantial evidence, including an associated viral illness for example, an upper respiratory infection or demonstration of seroconversion during the time of hearing loss. Cytomegalovirus. Cytomegalovirus (CMV) is a large, double-stranded DNA virus belonging to the herpesvirus family. Its name is derived from the typical appearance of infected tissues, containing massively enlarged cytomegalic inclusion cells. CMV is the most common congenital infection in the world. Of babies born in the...

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.

Infection Viral

Cytomegalovirus (CMV) is a double-stranded DNA virus in the herpesvirus family. CMV presents similarly to EBV and patients are commonly misdiagnosed with mononucleosis when acutely infected. CMV is transmitted by direct contact of mucosal surfaces, blood transfusion or organ transplant, maternal breast milk consumption, and rarely by aerosolized viral particles. Symptoms predominantly include fever, lymphadenopathy, and less commonly pharyngitis. Ulceration of the pharynx or esophagus may be present in severe cases, especially in immunocompromised patients with human immunodeficiency virus (HIV). CMV infection during pregnancy may result in fetal hearing loss, visual impairment, and diminished mental and or motor capabilities.

Trichina worm

Trichinosis is a zoonotic infection associated with the colonization of worms in muscles. It is often found in humans because of the consumption of uncooked or insufficiently cooked pork products (though other animals are also potential sources). Trichinella is the third most common worm that infects humans. They cause nausea, dysentery, puffy eyes, and colic. They also cause pain and more severe problems such as edema, cardiac and pulmonary problems, deafness, delirium, muscle pain, muted reflexes, nervous disorders, and pneumonia. Their natural hosts are flesh-eating animals, especially humans, pigs, rats, and other mammals. Humans are considered accidental hosts because, under normal conditions, the parasite ends its cycle that is, no other animals eat humans in order to transfer their larvae to other hosts. But concern for trichinosis is not as great today with improved pork production practices. Still, an estimated 5-6 million human infections are present at any one time in North...

Syphilis

Other head and neck manifestations of infection include sensorineural hearing loss in otosyphilis chancres of the oral cavity in primary syphilis and pharynx in secondary syphilis laryngeal chancres mucous patches or gummata encompassing the full spectrum of stages associated with syphilitic infection and numerous effects of congenital syphilis such as palatal defects, saddle nose deformities, and congenital deafness.

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.