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 Overview

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The Works of Beethovens Early Period and the Onset of Deafness

In the midst of this success, Beethoven showed the first signs of hearing loss. His first mention of deafness dates back to June 1801, when he noted that 'for the last three years my hearing has become weaker and weaker.' The cause has never been conclusively determined. In any case, it took years for his hearing to deteriorate fully, and it was not until his early to mid-forties that he was virtually completely deaf. In that span, encroaching deafness played a major role in shaping the direction of Beethoven's life and career. Obviously, it made socializing with friends more difficult and romantic relationships even more so. His capacity as a pianist would gradually decline to the point where he could no longer give a public performance. In the famous Heiligenstadt Testament, a letter written on 6 October 1802, Beethoven revealed depths of despair verging on suicidal. Fortunately, given the circumstances, one aspect of his life that did not deteriorate with his hearing was his...

Sudden Sensorineural Hearing Loss

Although most types of hearing loss are nonurgent problems, sudden sensorineural hearing loss (SSNHL) deserves special note because it is considered otologic emergency. Any patient complaining of sudden hearing loss requires prompt evaluation. An obvious cause such as cerumen impaction or middle ear fluid can be treated appropriately and routinely. If a cause is not identified, sudden sensorineural hearing loss should be suspected and prompt ENT referral arranged. SSNHL is thought to be secondary to vascular, thromboem-bolic, viral, or autoimmune causes. It may also be the result of ototoxicity. Without treatment, hearing returns in one third of patients, partial hearing returns in one third, and there is no improvement in the remaining third. Early intervention with oral corticosteroids (and possibly antivirals, directed at the herpesvirus Awad et al., 2008 ) appears to improve outcomes, although few controlled studies have been done. The Cochrane Collaboration believes that there is...

Sensorineural Hearing Loss

The cause of presbycusis is likely multifactorial, but ultimately the loss of cochlear hair cell function is thought to be the cause in most cases. Hair cell damage or loss can result from chronic noise exposure, genetic predisposition, and ototoxic medications. The hearing loss may also be caused by neurovascular injury from chronic conditions such as hypertension or diabetes, which can affect the cochlea or cochlear nerve. Hormonal conditions such as hypothyroidism should be considered, as should unusual conditions such as tertiary syphilis. Central auditory problems might be the cause, from dementia, cerebrovascular disease, or cerebrovascular accident (CVA, stroke). Although the term presbycusis implies sensorineural loss, conductive hearing loss should also be considered, including cerumen impaction, chronic OME, and otosclerosis (see Box 19-6).

Hearing Loss from Acoustic Energy

Excessive noise exposure is an important and usually preventable cause of hearing loss. Hearing loss can result from chronic or acute noise exposure, usually causing injury at the level of the cochlear hair cells. However, acute acoustic trauma can also cause injury to the tympanic membrane and middle ear structures. Chronic noise exposure may be recreational or vocational. The Occupational Safety and Health Administration (OSHA) has established guidelines for safe limits for acute and chronic noise exposure to prevent occupational noise-induced hearing loss. Exposure to noise of 90 dB or less is permissible for up to 8 hours per day. As the noise intensity increases, the permissible duration of exposure decreases. OSHA outlines procedures for hearing protection and monitoring. These standards also can help provide guidelines to minimize excessive recreational noise exposure. Recreational activities known to cause excessive noise include hunting or target shooting with firearms, use...

Hearing Loss in the Pediatric Population

Congenital hearing loss is often hereditary but may be secondary to an intrauterine insult or infection. Of the hereditary variety, the majority are autosomal recessive and nonsyndromic. Risk factors for congenital hearing loss include family history of hearing loss, facial abnormalities, ICU admission, history of meningitis, syndromes known to be associated with hearing loss, low Apgar scores at birth, medications known to cause hearing loss (e.g., aminoglycosides), elevated bilirubin, some prenatal maternal infections, or suspicion of hearing loss. Universal newborn hearing screening using otoacoustic emissions and auditory brainstem response allows early identification of impaired children. Intervention by age 6 months appears to improve language development. A temporal bone CT is often obtained to evaluate for inner ear malformations that would predispose the patient to further hearing loss with even mild head trauma. A genetics evaluation and counseling may be indicated....

Treatment of Hearing Loss

Individual treatments for specific causes of hearing loss vary greatly. This section gives a brief overview of options available to improve hearing in patients with the most common causes of hearing loss. Surgery is often performed for CHL. Myringotomy with or without tube insertion corrects hearing loss in cases of OME. The procedure is performed under brief general anesthesia in children or under local anesthesia for most adults. A tympa-noplasty is performed for tympanic membrane perforations and to reconstruct ossicles with a prosthesis. A stapedectomy with placement of a prosthesis is often a successful option in patients with otosclerosis. A variety of styles of hearing aids are used to rehabilitate hearing loss in patients with SNHL and CHL. The simplest (and least expensive) are larger, behind-the-ear aids with analog amplification of sound. The most complex (and most expensive) are completely-in-the-canal aids with programmable digital amplification. Several other types of...

Impact of Deafness on the Patient

It is estimated that there are more than 15 million persons in the United States with some degree of hearing loss that interferes with their ability to understand speech. About half of these individuals, 7.2 million, have bilateral hearing problems. Although persons older than 70 years of age account for 30 of all deaf individuals, there are more than 250,000 deaf children younger than 3 years of age. Since the late 1970s, the overall prevalence rate has increased substantially. To understand the impact of deafness on an individual, it is necessary to consider the age at onset, the severity of the loss, the rapidity of the loss, and any residual hearing. Persons with insidious or sudden hearing loss experience grief and depression. Consider, for example, the grief expressed in the quotation by Beethoven at the beginning of this chapter. The psychologic effects of deafness include paranoia, depression, withdrawal, irritability, and anxiety. Although it has not been proved, it appears...

Hearing Loss

Hearing loss results from an interruption in the transmission of sound or subsequent nerve impulses in one or more areas of the ear. Recognition and treatment of hearing loss are imperative unrecognized or untreated hearing loss may result in severe psychosocial ramifications in both adults and children. In the elderly population, hearing loss may lead to social withdrawal and depression. In the pediatric population, hearing loss may cause speech or cognitive delays. Hearing loss also has significant safety implications when it interferes with awareness of warning sounds (e.g., car horns, sirens, fire alarms). The four types of hearing loss follow 1. Conductive hearing loss (CHL) occurs when there is a failure of normal propagation of acoustic energy through the conducting portions of the ear, which include the external auditory canal and the middle ear. 2. Sensorineural hearing loss (SNHL) occurs from dysfunction of the inner ear, which may be caused by a failure of the generation of...

Translabyrinthine Craniotomy

Translabyrinthine craniotomy accesses the internal auditory canal (IAC), CPA, and posterior fossa by removal of the mastoid, the semicircular canals, and the vestibule of the inner ear. It provides the most direct approach to the CPA, eliminating the need for cerebellar or temporal lobe retraction, and provides excellent exposure of the entire IAC.3 Identification of the facial nerve as it exits the internal auditory canal facilitates nerve preservation. The main disadvantage of the trans-labyrinthine approach is that removal of the labyrinth results in hearing loss. This approach is best suited for medium to large lesions of the CPA that extend into the IAC, most commonly vestibular schwannomas or meningiomas. It is also appropriate for smaller lesions in patients with poor hearing in the affected ear. Removal of the cochlea with or without translocation of the facial nerve extends the translabyrinthine exposure to include the petrous segment of the internal carotid artery,

Infratemporal Fossa Approaches

Facial nerve translocation is often necessary, especially for type B and C variants, and may result in temporary paresis. Preauricular approaches to the infratemporal fossa, which do not require sacrifice of the external auditory canal and middle ear nor facial nerve translocation, are often appropriate for lesions that do not involve structures posterior to the intratemporal internal carotid artery.5

Laboratory Tests Audiogram

Any patient known to have or suspected of having a lesion of the lateral skull base or CPA should undergo formal audiomet-ric testing. Standard testing includes air and bone conduction pure tone thresholds, speech reception threshold, and speech discrimination scores. Lesions involving the middle ear typically result in a conductive hearing loss. Audiometrically conductive hearing losses manifest as elevated air conduction thresholds compared with bone conduction threshold (air-bone gap). Sensorineural hearing loss is caused by cochlear or auditory nerve dysfunction and demonstrates air and bone conduction thresholds elevated to the same degree. Lesions that involve the auditory nerve or auditory brainstem nuclei typically result in reduced speech discrimination scores out of proportion to their effect on pure tone thresholds. Any patient found to have an asymmetric sensorineural hearing loss or reporting unilateral tinnitus should be evaluated to exclude lesions of the IAC or CPA...

Synthesis of Thyroid Hormone

Thyroid peroxidase (TPO) is a membrane-bound glycoprotein that is localized to the apical membrane of the follicular cell the peroxidase reactions occur at the cell-colloid interface.24 TPO has now been cloned and has been shown to have a hydrophobic signal peptide at its aminoterminus and a hydrophobic region with the characteristics of a transmembrane domain near the carboxylterminus.23 This structure is consistent with TPO being a membrane-associated protein. The synthesis of thyroglobulin occurs exclusively in the thyroid gland, where homodimers are formed in the endoplasmic reticulum before being transported into the apical lumen of thyroid follicles.25 Defects in thyroglobulin synthesis usually cause moderate to severe hypothyroidism in association with low circulating thyroglobulin levels.25 A partial organification defect and goiter (with or without overt hypothyroidism) is associated with sensorineural deafness in Pendred's syndrome. Mutations in a putative sulfate...

Auditory Brainstem Response Testing

Auditory brainstem response (ABR) testing uses surface electrodes and a computer to record and average auditory nerve and brainstem potentials in response to click stimuli delivered to the ear. It is useful to screen patients with asymmetric sen-sorineural hearing loss or unilateral tinnitus for retrocochlear lesions. Although ABR is less sensitive than MRI with gado-

Local Or Intratumoral Chemotherapy

Benefit in primary CNS lymphomas but to date has not been associated with improved survival for patients with glioblas-toma. Significant toxicities have occurred, including seizures, hearing loss, new focal neurologic deficits, and coma. Drug transfer can potentially be increased further by iatrogenic BBB disruption using intra-arterial mannitol or RMP-7 (a bradykinin analogue) infusion. However, this approach also continues to be associated with increased toxicities in the face of limited clinical success.

Correlates of Psychosocial Adjustment

From research on the impact of condition severity and functional status also have found few differences (Wallander et al. 2003). However, if a child's condition involves the brain (e.g., epilepsy) or sensory systems (e.g., deafness or blindness), the risk of behavior problems and social adjustment difficulties is significantly greater (Mitchell and Quittner 1996 Walker et al. 1989). In addition, the level of

Beethovens Heroic Middle Period and the Transition to His Late Style

Compared to the preceding decade, the years 1813 to 1817 were far less productive, both in quality and quantity. Beethoven wrote only one or two works a year that have since entered the regular repertoire, all on a smaller scale than their predecessors. Alongside these works are a host of ephemera, including Wellingtons Sieg, Op. 91, probably the most vacuous thing Beethoven ever wrote (though he was clear about its meretricious origins) and the bombastic cantata, Der glorreiche Augenblick, Op. 136. Throughout much of this time, Beethoven was caught up in legal proceedings to gain custody of his nephew Karl, which surely took away from his time and energy for composition. Meanwhile, his almost total deafness continued to isolate him from most of society.

Beethovens Position in Music History

Beethoven's position in music history is absolutely central, both in terms of eminence and the standard chronology. Among composers his reputation is unparalleled. Chronologically, Beethoven is almost exactly in the middle of the historical span comprising the standard classical repertoire. He assimilated much of the earlier music in the tradition he inherited, bringing unsurpassed technical mastery and experimentation to the intense expression of personal emotions, and in the process reaching one of the highest summits of that tradition. His impact on later composers, for over a century, was almost immeasurable. Today, beyond his eminence in music, Beethoven is a universal popular icon his name, melodies, struggle with deafness, and even his unkempt hair are known to virtually every person in the world with access to print or other media.

Expertise acquisition and motivation

However Beethoven's basic intellectual capacity is conceptualized, it would have resulted in nothing without the willingness to channel his energy into the domain of music and work extremely hard at it, even in the face of major adversity, such as increasing deafness (In line with some conceptualizations of high ability, I include motivation as an intrinsic component of expertise acquisition). One vital basis of Beethoven's achievement was his early and continued acquisition in the domain of music. Numerous studies support the view that immense amounts of domain-specific knowledge are critically important for high-level performance and creativity. For instance, individual differences in conservatory-level music performance ability are largely attributable to the total amount of 'deliberate practice' engaged in. Similarly, even the greatest classical composers follow the so-called 'ten-year rule,' whereby even great creators require an apprenticeship of at least ten years of intensive...

Alexias and Agraphias

This disorder often overlaps with Wernicke's aphasia. In those cases, the patients often have a paraphasic output, impaired naming, and repetition, as well as disturbed written comprehension (word blindness) more than auditory comprehension (word deafness). Associated findings include right hemianopia or superior quadrantanopia, elements of Gerstmann's syndrome, and right-sided sensory loss. Most often, there is partial recovery, permitting only limited reading comprehension.

General Management Goals

Treatment should also include addressing complications of speech and language disorders. Depression is associated with left hemisphere injury, particularly in the deep frontal regions, and is associated with Broca's aphasia, global aphasia, or subcortical aphasia with anterior extension. y , y Treatment of depression includes support and encouragement, concentration on domains in which the patient succeeds, and antidepressant medication. Paranoid and agitated behavior is occasionally observed in patients with posterior aphasic syndromes, particularly those with Wernicke's aphasia and pure word deafness '571 management consists of antipsychotic drugs in a low dose. Finally, patients with associated bulbar or pseudobulbar palsy are at increased risk for aspiration and secondary lung infection and should be monitored closely and considered for percutaneous gastric feeding tube.

Emergency Department Treatment and Disposition

Patients should follow up in 10 to 14 days or return if symptoms persist or worsen after 48 hours. Refer patients who have significant hearing loss, have failed two complete courses of outpatient antibiotics during a single event, have chronic otitis media (OM) with or without acute exacerbations, or have failed prophylactic antibiotics to an otolaryngologist for further evaluation, an audiogram, and possible tympanostomy tubes.

Single Gene Inheritance

Some single gene, inherited characteristics are determined by genes located on the sex chromosomes. Males have XY sex chromosomes and females have XX sex chromosomes. The vast majority of sex-linked attributes are produced by recessive genes that are found only on X chromosomes, most often affecting males. Why is that With XY sex chromosomes, males are more susceptible to sex-linked traits because they don't have an additional X chromosome to carry an alternative version of the gene. Of the more than 100 sex-linked characteristics, many are disabling. These include hemophilia, two kinds of muscular dystrophy, and certain forms of deafness.

Otitis Externa Clinical Summary

Otitis externa (OE), or swimmer's ear, is an inflammation and infection (bacterial or fungal) of the auricle and external auditory canal (EAC). Typical symptoms include otalgia, pruritus, otorrhea, and hearing loss. Physical examination reveals EAC hyperemia and edema, otorrhea, malodorous discharge, occlusion from debris and swelling, pain with manipulation of the tragus, and periauricular lymphadenopathy.

The neurological neuropsychological behavioural and emotional consequences of TBI

The most common neurological symptoms following TBI include headache, pain, nausea, dizziness or vertigo, unsteadiness or poor coordination, tinnitus, hearing loss, blurred vision, diplopia, convergence insufficiency, increased light and noise sensitivity, and altered sense of taste and smell. TBI has been noted to cause injury to each of the cranial nerves (Russell, 1960) with the concomitant disruption of the various sensory and motor functions of the head. Waddell and Gronwall (1984), for example, have noted significant increases in the sensitivity to light and sound stimuli following mild TBI.

Diseases of the Islamic World

The population lived in the countryside at a subsistence level and often at the mercy of nomadic depredations. The medical works have the serious disadvantage of being largely nonclinical and highly derivative of classical medical texts, and the anecdotes of renowned physicians are frequently apocryphal. Yet major features of a pathological tableau do emerge, in which illness is inextricably tied to poverty as both cause and effect. Blindness is conspicuous, particularly in Egypt the result of a number of diseases, blindness seems to have afflicted a large percentage of the population, and the blind were traditionally employed as Koran reciters in mosques. Deafness was often congenital, and mutism was associated with nervous disorders. Paralysis, epilepsy, and mental disorders are frequently described in the medical and nonmedical texts, which include surviving magical incantations and prayers directed against demonic possession.

Directed Neurological Examination Sensory Function Of Cranial Nerve V

Involving the trigeminal motor nucleus such as motor neuron disease may produce fasciculations in association with muscle weakness and atrophy. One useful sign is the presence of masseteric spasm and contracture in association with ipsilateral hearing loss, facial numbness, diminished corneal reflex, and paretic facial muscles in patients with tumors infiltrating the dorsal pontine tegmentum. y , y

Preganglionic Syndromes

Trigeminal nerve compression can occur in the area between the brain stem nuclei and the gasserian ganglion, specifically within the brain stem itself or in the cerebellopontine angle. Patients can present with reduced facial sensation in association with poor hearing, nystagmus, limb ataxia, facial weakness, and a diminished corneal reflex. Common lesions in this area include tumorsy such as acoustic or trigeminal neuromas,y meningiomas, metastatic cancers, carcinomatous meningitis, and invasive nasopharyngeal carcinomas, inflammatory disorders such as sarcoidosis, or infectious processes such as mycobacterial (especially tuberculosis), fungal (candidal, histoplasmotic), parasitic, and bacterial organisms. Traumatic injury to this region may also result in sensory loss or motor deficits. Extensive brain stem and cerebellar signs may be evident from lesions in the cerebellopontine angle.

Gasserian Ganglion Syndromes

Numerous pathological processes occurring within the middle cranial fossa can result in trigeminal dysfunction by affecting the gasserian ganglion. In children, osteitis of the petrous apex following suppurative otitis media or mastoiditis, which leads to inflammation and infection affecting the trigeminal ganglion, may result in Gradenigo's syndrome. The syndrome is characterized by facial pain, headache, or sensory loss and a sixth cranial nerve palsy, facial palsy (due to seventh nerve involvement), and deafness (due to eighth nerve involvement). The pain is described as boring or throbbing, worse at night. Pain is aggravated by jaw or ear movement. It has been hypothesized that some of the dysesthetic sensation patients experience before or during episodes of Bell's palsy may reflect involvement of the trigeminal ganglion or nuclei in the brain stem. y A benign, self-limited trigeminal sensory neuropathy has been reported in children 7 to 21 days following a nonspecific febrile...

Associated Neurological Findings

The optic discs should be visualized for signs of increased intracranial pressure or stigmata of systemic conditions like hypertension or diabetes. Direct and consensual pupillary reflexes and extraocular movements, especially abduction of the globe (CN VI), should be evaluated. Facial sensation in the three divisions, as well as motor function of the muscles of mastication, should be checked to assess if CN V is also involved, as in a cerebellopontine lesion. Audition should be evaluated with tuning forks, looking for ipsilateral sensorineural or conductive hearing loss. If nystagmus is present, its direction and other characteristics should be documented. Unilateral vestibular weakness should be tested with the Mittelmeyer test (the patient marches in place with eyes closed and hands outstretched there is a tendency to turn toward the side of vestibular weakness). Palatal sensation and movement, vocal cord motion, trapezial strength, and tongue function should be...

History And Definitions

Hearing loss consists of a reduction, relative to normal standards, of the ability to perceive sound. Three subtypes are recognized conductive, sensorineural, and central. In conductive hearing loss, mechanical transmission of sound into the sensory receptors in the cochlea is impaired. In sensorineural hearing loss, there is a loss of function in the sensory receptors in either the cochlea or the auditory nerve. In central hearing loss there is a lesion in the brain stem or auditory cortex. 1855, developed the use of tuning fork tests in the diagnosis of middle ear disease. Meniere, in 1861, described a disorder of increased pressure in the inner ear, a disease that now bears his name. Barr, in 1901, described the use of a ticking watch to quantify hearing loss. Barany received the Nobel Prize in 1909 for his work describing the caloric test. Fletcher in 1923 described the electric audiometer. Bekesy received the Nobel Prize in the 1950s for his discovery of the traveling wave...

Table 122 Ototoxic Medications

Unilateral hearing loss Unilateral hearing loss Unilateral hearing loss Hearing loss Hearing loss Hearing loss nuclei in the brain stem. The superior olive is concerned with sound localization, based in interaural differences in sound timing and intensity. The superior olive is also an essential part of the stapedius reflex, which is a protective reflex in the middle ear. The simplest stapedius reflex arc involves the spiral ganglion neurons, the cochlear nucleus, superior olive, and facial nerve nucleus. Output from the superior olive joins crossed and uncrossed axons from the cochlear nucleus to form the lateral lemniscus. This pathway ascends to the inferior colliculus. Because the lateral lemniscus contains second order neurons from the cochlea and third and fourth order neurons from the superior olive, it contributes to three waves of the auditory brain stem response (described later). The tonotopic arrangement of the cochlea is maintained in the cochlear nucleus, lateral...

Evaluation Guidelines Tables125 and 126

Central hearing disorders Peripheral hearing loss Other Tests. The audiogram is the single most useful test in the patient with a hearing disorder and or vertigo. In hearing disorders, the audiogram is crucial in defining the degree and type of loss. In patients with vertigo, abnormalities in the audiogram usually narrow the differential diagnosis down to otological vertigo. Accompanying the audiogram is a battery of related measures. The tympanogram measures middle ear pressure, and tympanometry is helpful in identifying a perforated ear drum or middle ear infection. Acoustical reflexes measure the stapedius and tensor tympani reflex-generated ear drum movement in

Syndromes Primarily Involving Vestibular Function

Similar symptoms may be seen with other etiologies. Meniere's disease is usually recognized by the episodic pattern (see later). Herpes simplex virus infection of the vestibular nerve is recognized by a combination of ear pain and the presence of vesicles on the external canal. Acoustic neuroma (discussed later) is recognized by a slower course and the occurrence of hearing loss. Vascular disorders such as a labyrinthine artery infarction are generally impossible to exclude, and their diagnosis is suggested by an identical symptom complex combined with vascular risk factors. Acoustic neuromas and other tumors such as meningiomas, which can appear at the cerebellopontine angle, usually display asymmetrical sensorineural hearing loss. Usually, patients in the fifth or greater decade present with mild vertigo or ataxia, accompanied by a significant asymmetrical hearing loss. Generally, there are no other physical examination abnormalities, and facial and sixth nerve functions are...

General Considerations

To illustrate this concept, consider tinnitus as an example. Tinnitus, the name given to a sensation of sound in one or both ears, commonly accompanies deafness. When tinnitus is present, there is nearly always some degree of hearing loss. Conversely, when there is no appreciable hearing loss, there is rarely tinnitus. However, children who are born deaf do not complain of tinnitus.

Proximal Peripheral Lesions

The glossopharyngeal and vagus nerves pass through the area of the cerebellopontine angle, formed by the junction of the pons, medulla, and cerebellum, before exiting the skull through the jugular foramen. In this area, both nerves may be compromised by an expanding mass lesion, most commonly a schwannoma originating from the vestibular portion of the eighth cranial nerve within the internal auditory canal. The syndrome of a cerebellopontine angle tumor generally begins as tinnitus with hearing loss, and dysequilibrium or frank vertigo, which may be episodic. As the tumor expands, the fifth cranial nerve becomes involved, resulting in ipsilateral facial pain and numbness and loss of the corneal reflex. The cerebellum or cerebellar peduncles may become compressed, producing ataxia. Unilateral impingement upon the vagus nerve causes mild hoarseness and asymmetrical elevation of the soft palate. Schwannoma arising from the glossopharyngeal nerve is a rare occurrence, but, interestingly,...

Review of Specific Symptoms

Hearing loss Hearing Loss Hearing loss may be unilateral or bilateral and may develop slowly or occur suddenly. For any patient with a hearing loss, ask the following questions Is the hearing loss in one ear '' For how long have you been aware of a loss of hearing '' ''Was the loss sudden '' ''Is there a family history of hearing loss ''

Auditory Acuity Testing

Testing for auditory acuity is the next part of the physical examination. The easiest method of testing for a gross hearing loss is for the examiner to occlude one external canal by pressing inward on the tragus and to speak softly into the other ear. The examiner should hide his or her mouth to prevent lip reading by the patient. The examiner should whisper words such as ''park,'' ''dark,'' or ''daydream'' in the nonoccluded ear and determine whether the patient can hear them. This procedure is then repeated with the other ear. Asking a patient whether he or she hears a ticking watch held to the ear is generally unhelpful because the patient knows what to expect. The use of tuning fork testing for hearing loss is more accurate and should be performed regardless of the results of the whisper test. Although several tuning fork frequencies are available, the best for evaluating hearing is the 512-Hz fork.* A tuning fork is held by its stem, and the tines are briskly struck against the...

International resources in occupational toxicology

The American College of Occupational and Environmental Medicine (ACOEM) was founded in 1916. The College periodically issues position papers and committee reports that set practice guidelines for a variety of workplace environmental settings. These position papers committee reports cover topics such as spirometry, mould, environmental tobacco smoke, noise-induced hearing loss, multiple chemical sensitivities, workplace drug screening, confidentiality of medical information, depression screening, and reproductive hazards. In their 2005 position paper on toxicology these topics were identified as a core content of occupational and environmental medicine.

Vestibular Neuronitis

Distinguishing between vestibular neuronitis and bacterial labyrinthitis or labyrinthic ischemia is important. The diagnosis of bacterial labyrinthitis is based on hearing loss and otitis media or meningitis, and labyrinthic ischemia can be distinguished by hearing loss plus associated neurologic symptoms with a history of vascular disease.

Traumatic Tympanic Membrane Perforations

Traumatic perforation of the tympanic membrane may result from barotrauma (water skiing diving injuries, blast injuries, blows to side of head), ear canal instrumentation (cotton-tipped applicators, bobby pins, paper clips, cerumen curettes), or otitis media (see earlier discussion). The patient usually complains of acute pain that subsides quickly, associated with bloody otorrhea. Severe vertigo can occur but is transient in most cases. Persistent vertigo suggests inner ear involvement (perilymphatic fistula). Hearing loss and tinnitus are also common. Findings may include fresh blood in the canal and around the perforation. Any medial canal clots or debris should not be removed or irrigated except under microscopy. Secondary bacterial infection may require treatment with ototopi-cal antibiotics. Topical fluoroquinolones are safe for use in the middle ear. Audiologic evaluation is necessary to rule out sensorineural hearing loss. If a tuning fork examination indicates sensorineural...

Barotrauma and Barotitis

Changes in altitude while flying (or scuba diving) can lead to rapid changes in middle ear pressure, leading to accumulation of serous middle ear fluid or blood. Symptoms may include aural fullness, otalgia, and conductive hearing loss. In most cases the fluid is resorbed, although this may take several weeks. Autoinflation maneuvers (popping the ears) may hasten recovery. Oral and topical decongestants, nasal steroid sprays, or a short course of corticosteroids may be helpful. Antibiotics are indicated only if there are signs of infection. If fluid persists or is troublesome to the patient, a myringotomy allows the fluid to be drained. Tympanos-tomy tube insertion may be indicated for persistent middle ear fluid. Rarely, a rapid change in middle ear pressure can lead to the creation of a perilymphatic fistula between the inner and middle ear. The patient complains of severe vertigo and hearing loss (sensorineural) (see Vertigo). Urgent ENT consultation is indicated.

Clinical Presentation

Signs and symptoms not attributable to the lipoma but secondary to the associated developmental anomalies. Approximately 30 of patients with supratentorial lipomas have a seizure disorder. The seizure disorder is reportedly often severe, is focal or generalized, and has an average onset age of 15 years. Recurrent headaches are a presenting symptom in 25 percent, and . . . 10 to 15 percent have behavioral problems and or mental retardation.23 Other reported signs and symptoms include fainting spells, vomiting, episodic leg weakness, blurred vision, sleepwalking, diencephalic disturbances such as adiposogenital dystrophy and hypothermia, and even transient ischemic attacks.6 Presenting signs and symptoms of patients with infratentorial lipomas may include hydrocephalus, cerebellar ataxia, motor weakness, sleep apnea, and cochleovestibular symptoms such as hearing loss, vertigo, otalgia, trigeminal neuralgia, hemifacial spasm, and tinnitus.6,19,23

Inflammatory Disorders

Mumps is a relatively common cause of painful unilateral or bilateral parotid gland enlargement in children. It is caused by a paramyxovirus, and diagnosis is confirmed by elevation of antibodies to the S and V virus antigens or by isolation of the virus in the urine. The incubation period is 2 to 3 weeks, and infection lasts 7 to 10 days. Treatment is conservative, with close follow-up to observe for possible complications such as pancreatitis, meningitis, orchitis, and hearing loss.

Congenital Pulmonary Stenosis

Pulmonary artery and its branches22,37'72'79'102,112 and patent ductus arteriosis37,79 are features of what came to be known as the rubella syndrome. Maternal viremia is a prerequisite for placental and fetal infection during initial exposure to rubella.37 Maternal rubella can have serious noncardiac effects, including spontaneous abortion, stillbirth, mental retardation, cataracts, and deafness.37 Fetal risk is small when infection occurs later than the 16th

Cross Modal Plasticity

Cortical representations that have been deprived of their normal modality-specific sensory input have become responsive to other sensory modalities. Sensory deprivation due to deafness or blindness from the time of birth leads to some remarkable organizational changes detected by TMS, PET, and fMRI. Cross-modal reorganization following sensory deprivation has also been demonstrated later in life. This phenomenon, if better understood, could lead to retraining and biologic interventions for patients with sensory and perceptual impairments.

Development Of The Dorsal And Ventral Visual Streams

In our laboratory, we have studied the effects of congenital deafness on visual functions. Our functional MRI studies have shown that auditory deprivation affects processing of visual motion. Congenitally, genetically deaf and hearing participants were presented with a moving random-dot field and were asked to attend to changes in the brightness of the dots and changes in the velocity of the motion (Bavelier et al., 2000, 2001). When attending to the center of the flow field, the two groups produced similar activation. By contrast, when attending the periphery of the flow field, deaf adults displayed more activation within dorsal stream area MT, the posterior parietal cortex (PPC), and the superior temporal sulcus (STS) than did hearing

Children Eligible for Services

Mental retardation, hearing impairments (including deafness), speech or language impairments, visual impairments (including blindness), serious emotional disturbance (hereafter referred to as emotional disturbance), orthopedic impairments, autism, traumatic brain injury, other health impairments, or specific learning disabilities and who, by reason thereof, needs special education and related services (Pub. L. No. 105-17, 602, 111 Stat. 43 1997 ). (2) Deaf-blindness means concomitant hearing and visual impairments, the combination of which causes such severe communication and other developmental and educational needs that they cannot be accommodated in special education programs solely for children with deafness or children with blindness. (3) Deafness means a hearing impairment that is so severe that the child is impaired in processing linguistic information through hearing, with or without amplification, that adversely affects a child's educational performance. (5) Hearing...

Functional Results and Rehabilitation

'Average hearing loss at 1 to 3 kHz. 'Average hearing loss at 1 to 3 kHz. Cross rehabilitation equipment can be mounted to spectacles in case of unilateral deafness and allows the patient, via a receiver on the deaf side and a loudspeaker on the hearing side, to perceive noises and voices on the deaf side. Auditory brainstem implants are designed for patients with NF2 who have bilateral hearing loss caused by bilateral destruction of the cochlear nerves from schwannomas. Implants initially give patients some perception of environmental sounds patients then relearn to understand syllables, whole words, and finally sentences. With implants and lipreading, they achieve an understanding of 40 to 100 of conversation of an unknown context. 'Preservation in H5 signifies recovery from functional deafness to auditory function.

Outcome and Quality of Life

On close examination, head and neck pain are found to originate from occipital nerve irritation (rare) or preexisting cervical osteochondrosis. Tinnitus is less common in patients with some preserved hearing, whereas it is increased or more irritating to patients with postsurgical deafness. Balance disturbances resulting from the unilateral loss of vestibular nerve

Which regimen should be considered standard of care for goodrisk metastatic germ cell tumor

Underlying pulmonary disease, smoking, and cumulative bleomycin dose, there may be reason to choose four cycles of EP and avoid bleomycin. It should be remembered, however, that the overall risk for BIP with 270 units of bleomycin is low, so avoidance of bleomycin in young individuals who have normal renal and pulmonary function is not warranted. In patients who have underlying peripheral neuropathy or hearing loss in whom minimizing cisplatin exposure might be advantageous, three cycles of BEP may be preferred to avoid the extra cycle of cisplatin.

Facial Nerve Schwannomas

The geniculate ganglion level grow into the middle fossa, and those with proximal origin extend into the internal auditory meatus and cerebellopontine angle. Mean age of patients is approximately 40 years.8,10 Facial palsy occurs in most, but not all, cases it can be absent in up to one quarter. Severity of facial weakness ranges from mild paresis to total palsy. It is usually progressive, often proceeded by periods of facial twitching. Sensorineural deafness is usually present. It can be severe or total. However, it is conductive in some cases and rarely the patient can have intact hearing.8 Other symptoms may include vertigo, tinnitus, or ear pain.10 There is a long interval between onset of symptoms and diagnosis. Facial nerve innervates the muscles of facial expression. It also innervates the stapedius muscle, salivary and lacrimal glands, and mucus glands of the nose, nasopharynx, and pharynx. It conveys taste sensation from the anterior two thirds of the tongue and carries...

Posterior Fossa Meningiomas

Posterior fossa meningiomas account for less than 10 of total intracranial meningiomas. Half of these are in the cerebello-pontine angle 40 occur at the tentorium or cerebellar convexity. Approximately 9 are clival and 6 occur at the foramen magnum. Petroclival meningiomas arising medial to the trigeminal nerve have a higher rate of surgical morbidity (Figure 45-11).12 Meningiomas of the petrous pyramid can cause hearing loss, facial pain, and facial weakness. Headaches and ataxia are common with larger lesions. Primary jugular foramen tumors are probably one of the rarest subgroups of meningiomas. The most common presenting symptoms are hearing loss, swallowing difficulties, and weakness in the trapezius or sternocleidomastoid muscles. The senior author has successfully resected these tumors through either a suprajugular, transjugular, or retrojugular approach.5 The most common dysfunction following surgery was transient deficit in ninth and tenth cranial nerve functions. Transient...

Hearing and Vision Screening

Early detection and intervention for hearing and vision deficits are important for maximal long-term functioning. Without appropriate opportunities to learn language, children with significant hearing deficits fall behind peers in terms of communication, cognition, reading, and social-emotional development, with long-term effects on educational attainment and adult employment (AAP Joint Committee on Infant Hearing, 2007). It is now recommended that all infants be screened for hearing loss by 1 month of age, regardless of risk factors. Those who do not pass the screening should have a complete audiologic evaluation by 3 months of age, and those with confirmed hearing loss should receive appropriate treatment by 6 months to ensure optimal outcome. Regardless of the outcome of newborn screening, ongoing surveillance of hearing status is recommended. Developmental delays and other risk factors (Box 23-4), particularly in language, as well as the presence of parental concern about hearing,...

Evaluation of Developmental Delay

Physicians to start the workup while waiting for a specialist appointment. Results of the workup may facilitate more specific referrals. Even if not globally delayed, all children with language delay should have a formal audiology assessment to rule out hearing impairment.

Clinical Manifestations

Upper and lower cranial nerve palsies. Permanent or intermittent diplopia is the first symptom in most patients, generally the result of compromising of the sixth cranial nerve.1,8,16 Other symptoms include those related to other cranial nerve palsies, such as decreased visual acuity, facial numbness, facial weakness, hearing loss, dysphasia, dysarthria, hoarseness, and difficulty with speech and swallowing, as well as symptoms of brainstem or cerebellar compression such as dysmetria, gait ataxia, motor weakness, and memory disturbances. Local tumor extension into the retropharyngeal space or into the nasal cavity may occur, and nasopharyngeal symptoms, such as nasal obstruction or discharge, eustachian tube obstruction, throat fullness, dysphasia, or epistaxis, may occur, occasionally being the only symptoms.1,16,20,21 Physical examination often discloses optic nerve dysfunction or extraocular palsies, particularly when the tumor involves the upper clivus.8,20,21 Cranial nerve...

The superior cerebellar artery SCA

Nystagmus (middle and or superior cerebellar peduncle, superior cerebellum and vermis), ipsilateral Horner's syndrome (descending sympathetic tract), contralateral fourth palsy (IV nucleus), and contralateral thermoalgesic sensory deficit (spinothalamic tract) may be present. Other signs have been described, such as ipsilateral chorei-form abnormal movements or palatal myoclonus (superior cerebellar peduncle interrupting the denta-torubral pathway), sleep abnormalities, and partial contralateral deafness (lateral lemniscus). Given its close relationship to the distal basilar artery, SCA strokes are very frequently embolic (from an arterial or cardiac source).

Clinical Manifestations and Pathology

In prenatal infections most infants are born without clinical evidence of disease, although some 10 to 15 percent may show microcephaly, retardation of growth or mental development, hepatosplenomegaly, jaundice, and calcifications in the brain. There may be abnormalities in liver function tests and in hematopoiesis. Some 10 to 30 percent of infants with symptomatic disease die in early life. Evidence of involvement of the central nervous system can develop in the early years of life, even though the child may appear normal. The evidence is manifested as impaired intellect, neuromuscular abnormalities, chorioretinitis, optic atrophy, or hearing loss.

Diagnosis Clinical Presentation

The clinical characteristics of a glomus jugulare tumor depend on its locally invasive behavior, its anatomic extension, the size of the tumor, and whether it secretes neuropeptide hormones. Most commonly, patients have hearing loss and pulsatile tinnitus or dizziness. Hearing loss is usually unilateral and results from invasion of the middle ear. It can be conductive if the ear canal is obstructed or sensorineural if the cochlea or labyrinth is invaded, and is often accompanied by dizziness. Pulsatile tinnitus occurs in association with a highly vascular lesion, which is seen through an otoscope as a pulsatile reddish-blue mass beneath the tympanic membrane. Occasionally, patients develop otorrhea spontaneously or after a biopsy.

Abnormal Findings and Clinical Uses of the BAEP

The BAEP is also useful in assessing the integrity of the brain stem (see Fig,, 24-13, ). Although the auditory pathways have a bilateral course, a unilaterally abnormal BAEP nevertheless suggests an ipsilateral, structural brain stem lesion. The presence of normal BAEPs in comatose patients suggests either that the coma is due to bihemispheric disease or that it relates to metabolic or toxic factors abnormal BAEPs in this context imply brain stem pathology and a poorer prognosis than otherwise. When coma is due to brain stem pathology, the BAEP findings help in localizing the lesion alteration of wave V suggests midbrain dysfunction, and alteration of waves I to III indicates a lesion in the lower brain stem. y In patients with brain death, the BAEP should be absent apart from wave I and the early part of wave II, which are generated peripherally. In many patients with suspected brain death, however, all BAEP components, including wave I, are absent, and it is then not possible to...

Perception and Construction Abilities

Speech-Sound Perception Test.y This test is part of the Halstead-Reitan Battery. Patients listen to 60 nonsense syllables on a tape and must choose among four options of printed versions of the sounds. One problem with this test is that individuals who have high-frequency hearing loss tend to perform poorly.

Eugenics Discrimination And Stigmatization

While there may be strong logical arguments to the effect that genetic screening does not necessarily discriminate against people with disabilities, nevertheless as long as the point is perceived in this way, the potential for adverse consequences is considerable. One of the most significant implications of genetic screening is the potential effect on our very understanding of concepts of normality and disability. The conceptual and political contest over our understanding of deafness, for example, becomes intensified. This may be the case even in relatively noncontroversial contexts Normality is a relative concept, and any health-related examination therefore includes a risk of the examinees feeling or being felt to be thought of as abnormal or just plain ill'' (Danish Council, p. 60).

Noninfantile Refsums Disease

This lipidosis, also known as heredopathia atactica polyneuritiformis, is a familial disorder characterized clinically by retinitis pigmentosa, peripheral neuropathy, cerebellar ataxia, and high protein levels in the cerebrospinal fluid without pleocytosis. Cardiac involvement and nerve deafness occur in almost all patients as well. Ihe accumulated material is phytanic acid. This condition is distinct from the peroxisomal disorder of infantile Refsum's disease, in which phytanic acid accumulation is not the primary metabolic defect but a secondary result of peroxisome assembly.

Temporal Bone Syndrome

Conductive hearing loss is the most common manifestation of temporal bone metastasis. It is present in approximately 30 to 40 of symptomatic patients and is almost always the result of dysfunction of the eustachian tube with secondary serous otitis media.9,26,61 Sensorineural hearing loss, if it occurs, is usually due to involvement of the cochlear fibers in the internal auditory meatus.61 Maddox emphasized the triad of symptoms of otalgia, periauricular swelling, and facial nerve paresis as being the most suspect for malignant involvement of the temporal bone.41 He reported an incidence of facial nerve paralysis of 34 in his series. Schuknecht et al also reported a high incidence of facial palsy.63 Saito et al found that only 50 of patients with invasion of the facial canal manifested facial paralysis, although 100 of those who had tumors extending beyond the epineural sheath had complete paralysis.60 Much less common findings are otorrhea, vertigo, tinnitus, or a middle ear...

Myoclonic Epilepsy and Ragged Red Fibers Syndrome

The classic presentation is usually in late childhood, although it may begin in early adulthood, with progressive myoclonic epilepsy, ataxia, and action-induced polymyoclonus. Weakness and hypotonia due to a mitochondrial myopathy and a progressive dementia follow. Hearing loss may be associated. This presentation resembles the historical Ramsay Hunt syndrome of dysergia cerebellaris myoclonica.

Mitochondrial Encephalopathy Lactic Acidosis and Strokelike Episodes

Patients may present sporadically or in maternal pedigrees, in infancy, childhood, or adulthood. The overall course is of a progressive degenerative disease with strokelike episodes and a mitochondrial myopathy. Various symptoms appear singly or in combination and include hemiplegia, sudden cortical blindness, hemianopia, episodes of confusion and hallucination with fever, aphasia, migraine headaches in a maternal lineage preceding strokelike episodes, and maternally inherited diabetes mellitus type II and deafness. Multisystem complaints and signs include myalgia, fatigability, weakness, ophthalmoplegia, pigmentary retinal degeneration, cardiomyopathy, cardiac conduction defects and block, dementia, deafness, ataxia, myoclonus, seizures, lactic acidosis, and proximal renal tubule dysfunction.

Chromosomal Anomalies

In addition to the typical phenotypic features of the syndrome, associated congenital cardiac and gastrointestinal abnormalities may be present. A third to a half of patients with Down's syndrome have congenital cardiac defects, of which one third are endocardial cushion defects, and the remainder are ventricular septal defects. Tetralogy of Fallot and atrial septal defects also occur, and there is an increased incidence of moyamoya disease. More than half of patients have bilateral hearing loss, of which many cases are attributable to anomalies of the inner and middle ear. Malformations of the gastrointestinal tract, including intestinal atresia and imperforate anus, occur in about 5 to 7 percent of patients, and there is a reported increased incidence of Hirschsprung's disease. Although abnormalities of T-lymphocyte function have been reported, no specific relationship of these to the infection rate has been established. Other associated abnormalities include gastroesophageal...

Call Your Doctor Now

Deterioration in the ability to hear some or all sounds may come on gradually over a period of several months or years or may occur suddenly over a matter of hours or days. In many cases, hearing loss is the result of an ear infection or a wax blockage and can be treated easily. Hearing loss is also a common feature of aging. However, if you suddenly develop severe hearing loss in one or both ears for no obvious reason, always consult your doctor.

Sequelae Complications And Their Management

Rehabilitation of patients with chronic aspiration may necessitate surgical procedures such as vocal cord medialization, laryngoplasty, tracheostomy, feeding gastrostomy and occasionally even laryngectomy. Treatment for tumors involving the orbit or its surroundings has the potential for impairment of vision either due to direct involvement of the globe or due to dysfunction of the optic nerve and nerves to the extraocular muscles. Radiation therapy fields must be planned very carefully to avoid exposure of the optic nerve or chiasm, and complications can be minimized by using accurate delivery techniques such as IMRT. Facial nerve paralysis can result in exposure keratitis which may be avoided by anticipating the need for gold weight implants or tarsorrhaphy. Radiation therapy, especially in young individuals, can cause cranio-facial growth defects, cataracts, hearing loss, hypopi-tuitarism, and can also induce subsequent malignant tumors. Long-term multidisciplinary follow-up of...

Spinocerebellar Ataxia Type

Or optic atrophy, suggesting that retinopathy affects only the macula. All patients in whom the disease begins earlier (before the age of 40 years) have visual problems, starting either prior to or at the same time as the appearance of cerebellar ataxia. The majority of these patients have retinal degeneration, and some also have optic atrophy. Tendon reflexes are usually absent. A number of additional symptoms occur in less than half the patients and tend to be more frequent in patients with a long disease duration. These symptoms include gaze palsy, dysphagia, hearing loss, and muscle weakness. Dementia and basal ganglia symptoms are not typical features of ADCA-II. d , y

Treatment Goals of Therapy

The introduction of antibiotic therapy and vaccines has reduced dramatically the mortality associated with bacterial meningitis.19 Prior to these advances, bacterial meningitis was almost universally fatal, and those few patients who survived often suffered from debilitating residual neurologic deficits, such as permanent hearing loss. Although significant improvements have been made, the fatality rate of pneumococ-cal meningitis remains above 20 likely due to its occurrence in debilitated patient populations.

Facioscapulohumeral Muscular Dystrophy

Several other problems have been identified in FSH MD, particularly in patients with early onset and relatively rapid progression. Hearing loss in the 4000 to 6000 Hz range and retinal capillary abnormalities (Coat's disease) have been reported. Although it is not usually clinically apparent, abnormal AV node or intranodal conduction also occur.y

Patient Care and Monitoring

Monitor the patient's response to therapy (i.e., clinical signs symptoms and laboratory data), as well as the development of complications, including seizures and hearing loss. Dexamethasone therapy may reduce antibiotic penetration, so antimicrobial drug dosing may have to be increased (especially vancomycin) to achieve dequate CSF levels. Serum levels of vancomycin should be measured and doses titrated to ensure adequate CNS concentrations. Evaluate whether intraventricular or intrathecal antibiotics are indicated. 12. Arrange for patient follow-up after discharge from the hospital. Continue to monitor for neurologic sequelae for several months after completion of treatment, and educate the patient and family in this regard. Serious complications that may occur include, among others, hearing loss, hemiparesis, quadriparesis, muscular hypertonia, ataxia, seizure disorders, mental retardation, learning disabilities, and obstructive hydrocephalus.

Psychiatric Disorders That May Be Mistaken for Malingering

The somatoform disorders include hypochondriasis, pain disorder, body dysmorphic disorder, and conversion disorder. Conversion disorder may be the most likely somatoform disorder to be mistaken for feigning. In this disorder, individuals present with pseudoneurological deficits of voluntary motor or sensory function that typically fail to follow known anatomical pathways. Other conversion symptoms include aphonia, urinary retention, blindness, deafness, hallucinations, and seizures. Clinicians should be cautious in diagnosing somatoform disorders during initial evaluation and make reasonable efforts to ensure that medical illness has been ruled out, as sometimes medical illness later surfaces that explains symptoms (American Psychiatric Association 2000).

Hereditary Sensory and Autonomic Neuropathies

The complications of HSAN I may be of sudden, overwhelming onset, with ulcers first developing over pressure points on the foot. When these ulcers are neglected, they do not heal and are associated with the complications previously described. Phenotypes have been reported in which HSAN is associated with deafness, leg weakness and atrophy, burning feet, restless legs, and neuropathic atrophy. HSAN is a slowly progressive disorder that does not seem to decrease the lifespan. It cannot be emphasized enough that proper foot care can prevent complications that can lead to foot amputation and sepsis.

Neurofibromatosis 2

Molecular Genetics and Clinical Presentation. Neurofibromatosis type 2, also known as bilateral acoustic or central neurofibromatosis, is less common than NF1. NF2 occurs in 1 out of every 40,000 to 100,000 live births.351 Isolated in 1993, the NF2 gene is located on chromosome 22 (22q12.2 locus) and codes for a protein called merlin, or schwannomin.17-18,48 Merlin, or schwannomin, is expressed in fetal brain, kidney, lung, breast, and ovary. Its function is unclear but possibly involved with membrane stabilization of a cytoskeleton-associated protein and intercellular communication. The loss of merlin may lead to the elimination of intercellular contact inhibition, which could lead to tumor development.102 In 10 of cases, NF2 patients become symptomatic before the age of 10. Fifty percent of NF2 patients become symptomatic by age 30 and 90 by age 50.3 NF2 is characterized by schwannomas of the cranial nerve roots, particularly the vestibular portion of the eighth cranial nerve, as...

Lightning Injuries Clinical Summary

Lightning produces injury from high voltage, heat production, and explosive shock waves. Direct injuries include cardiopulmonary arrest, cardiac arrhythmias, and neurologic abnormalities such as seizures, deafness, confusion, amnesia, blindness, and paralysis. The patient may suffer contusions from the shock wave or from opisthotonic muscle contractions. Chest pain and muscle aches are common. One or both tympanic membranes rupture in more than 50 of victims. Cataracts are usually a delayed occurrence. Hematologic abnormalities including disseminated intravascular coagulation (DIC) have been described. Fetal demise may occur.

The Continuing Prominence of Goffman

While a classic statement that highlighted a key moment in the sociology of mental health, the scope of Goffman's book was broad, covering a range of substantive areas that produced a general perspective on the social consequences of difference. He considered the situation of being an orphan, facing hearing impairment or a wide spectrum of disabilities, engaging in criminal behavior, and holding identities from prostitute to revolutionary, in addition to having a mental illness.

Charcot MarieTooth Disorder

Charcot-Marie-Tooth (CMT) disorders, also known as hereditary motor and sensory neuropathy or peroneal muscular atrophy, represent an inherited group of neuropathies, some of whose genetic mutations have been characterized. These disorders are associated with diffusely enlarged peripheral nerves, most commonly involving the peroneal nerve. Prevalence of CMT disorder is 1 person per 2500 in the population, or approximately 125,000 patients in the United States. Patients with CMT usually have a slowly progressive degeneration of the muscles in the foot, lower leg, hand, and forearm, and a mild loss of sensation in the limbs, fingers, and toes. The first sign of CMT is generally a high-arched foot or gait disturbance. Other symptoms of the disorder may include foot bone abnormalities such as hammer toes, problems with hand function and balance, occasional lower leg and forearm muscle cramping, loss of some normal reflexes, occasional partial sight or hearing loss, and, in some patients,...

What Is A Hemispherectomy

Continuously over one hemisphere, together with frequent intractable seizures. Some of these patients have seizure discharges that cross the corpus callosum to the opposite side, causing interferences in the function of the opposite hemisphere as well. Often, cognitive function in these patients, as well as normal fine movement in the foot and hand on the side opposite the affected hemisphere, is severely handicapped. However, as patients in whom this condition is congenital grow up, they become remarkably mobile despite their affected extremities. These patients will often experience dramatic improvement with removal of the atrophic or poorly developed cortex that is causing seizure discharges but is not critical for significant useful function. Formerly, the entire cortical mantle was removed. However, this technique tended to cause hemorrhaging into the large, empty removal cavity that became filled with spinal fluid after the cortical excision. This hemorrhaging could lead to...

Clinical Features and Associated Findings

Features of the congenital rubella syndrome include intrauterine growth retardation, sensorineural deafness, cataracts, congenital glaucoma, patent ductus arteriosus, pulmonic stenosis, microcephaly, mental retardation, meningoencephalitis, purpuric rash, hepatosplenomegaly, and radiolucent bone densities. y In addition to these clinical signs, which are present at birth, children with congenital rubella syndrome may develop delayed-onset progressive rubella panencephalitis, a slow virus disease of the CNS that has many clinical similarities to subacute sclerosing panencephalitis caused by measles virus. y This syndrome may also follow childhood rubella in rare instances. y Symptoms typically begin between the ages of 8 and 19 years with deterioration in school performance and behavior sometimes associated with seizures. The neurological deterioration progresses to a global dementia with ataxia, spasticity of gait, dysarthria, and dysphagia. Progressive...

The Meaning Of Medical Futility

A series of court cases revealed sharp controversies among clinicians, patients, or families about what is medically useful or futile (L. M. Kopelman, 1995. J. Med. Philos. 20,109-121). These disputed cases include that of Nancy Cruzan, a woman in a coma or persistent vegetative state (PVS) whose family wanted her feeding tube removed Baby Doe, an infant with Down's Syndrome whose parents did not want surgery to correct anomalies incompatible with life (Kopelman et al., 1992) Baby L, with multiple disabilities including blindness, deafness, quadriplegia, and arrested development at the 3-month-old level whose mother insisted on maximal treatments Helga Wanglie, a woman in a PVS whose family said she wanted maximal treatment and

Treatment Desired Outcome

With regard to neurosyphilis, a reduction in neurologic manifestations is desired, which may include seizures, paresis, hyperreflexia, visual disturbances, hearing loss, neuropathy, or loss of bowel and bladder function. In late neurosyphilis, vascular lesions (meningovascular neurosyphilis) may also be observed thus, a reduction in the number of observed lesions is warranted. A diminution in CSF WBC (less than 10 x 103 mm3 10 x 109 L ) or protein levels (0.05 g dL 0.5 g L ) is also preferred.

Causes of Speech and Language Difficulties

The cause of speech and language difficulty actually may be a hearing loss or an abnormality in the structure of the mouth, larynx, or the throat that interferes with the production of proper speech. In many instances, however, there is no structural cause for a language or speech problem. When this is the case, these problems are related to the temporal lobe of the brain's left hemisphere, where speech is produced and modulated.

Acoustic Schwannoma and Trigeminal Neurilemmoma

Clinical Features and Associated Disorders. Acoustic neuromas are considered slow growing, but they grow in a physiologically eloquent area, the cerebellopontine angle, thus leading to subtle complaints that may evolve slowly over many years. Symptoms may progress slowly, although sudden presentations can be seen. Patients usually present with hearing loss (especially sound discrimination), tinnitus, loss of balance, nystagmus, loss of facial sensation, or loss of function of the facial muscles or the muscles of mastication. Pain is uncommon but may sometimes mimic trigeminal neuralgia. Cerebellar findings such as ataxia and gait abnormalities may be noted when extremely large tumors have extended into the cerebellum. In patients with trigeminal pain multiple sclerosis may be diagnosed initially only with further hearing loss and a lack of involvement of other areas of the nervous system will re-evaluation be conducted. Meningiomas are occasionally confused with neuromas. Rarely,...

Glomus Jugulare Carotid Body Tumors

Glomus jugulare tumors arise from the paraganglionic tissue surrounding the jugular vein in the area of the middle ear. These tumors most often present late in life, in the sixth or seventh decade, and may have a genetic predisposition. They are locally invasive and highly vascular. Although presenting symptoms include complaints similar to those typical of acoustic neuromas, with tinnitus or hearing loss, the key differentiating features of these tumors are pain and the presence of loud pulsations in the ear. Occasionally, blood may drain from the ear. On examination, the patient may have tumor visible in the ear canal and, with larger lesions, evidence of cranial nerve or cerebellar dysfunction. The differential diagnosis includes neuromas, cholesteatomas, meningiomas, vascular malformations, or metastatic disease.

Labyrinthitis Vestibular Abnormalities and Menieres Disease

Patients with labyrinthitis usually experience hearing changes, whereas those with vestibular neuronitis do not. A viral infection is the usual cause in younger patients while infarction becomes more likely in the older adult. Older-adult patients may recover more slowly and experience feelings of imbalance for several months. Treatment during the symptomatic period may include vestibular rehab exercises and pharmacologic agents such as meclizine, promethazine, or low-dose benzodiazepine (e.g., lorazepam). Meniere's disease should be suspected when an older adult reports recurrent episodes of vertigo, tinnitus, gradual development of low-frequency hearing loss, and in some cases ear fullness before onset of vertigo.

Tinnitus The Ring You Cant Answer

Ringing, buzzing, hissing, chirping, and whistling will it ever stop For an estimated 50 million Americans with tinnitus, commonly called ringing in the ears, it doesn't. I have lectured to hospital-based tinnitus support groups and heard the word about this incredibly annoying and aggravating condition. The ear noises can be intermittent or continuous, and the loudness also varies. Background noises of daily life often drown out the ringing or buzzing during the day. The worst time seems to be when you're trying to fall asleep in a quiet room. The noises can be so distracting that they interfere with your concentration, work, relationships, and sleeping patterns. For many, the personal distress creates anxiety about going to bed at night. Tinnitus is often associated with hearing loss, but it does not cause the loss, nor does hearing loss cause tinnitus. The cause of tinnitus is obscure.

Two Or More Of The Following

Headache in WG is due to sinusitis, pachymeningitis, or cerebral vasculitis. Multiple forms of cranial nerve problems occur. Exophthalmos, ophthalmoplegia, and optic nerve involvement due to granulomatous involvement may be a presenting symptom of WG. y Ocular symptoms are present in 15 percent of patients at onset and 52 percent of patients during the disease course. The most helpful diagnostic finding is proptosis (2 percent at onset, 15 percent overall), since it is highly suggestive of WG when associated with upper or lower airway disease. Proptosis is usually painful and leads to visual loss owing to anterior ischemic optic neuropathy in about half of affected patients. Extraocular muscle entrapment can cause diplopia. y Recurrent serous or suppurative otitis media may cause partial (33 percent) or total unilateral (1 percent) or bilateral (1 percent) hearing loss. y Multiple cranial neuropathies rarely occur as a result of pachymeningitis. y

Progressive Systemic Sclerosis

Clinical Features and Associated Disorders. Some patients have a relatively benign form of the disorder limited to cutaneous changes in the distal extremities. In the generalized form of the illness, cutaneous abnormalities may predominate, but systemic symptoms, including generalized malaise, are common. Vascular changes in the hands progress from Raynaud's phenomenon to frank necrosis and induration and fibrosis of the skin (sclerodactyly) with atrophy of the underlying muscle. Rigidity of ligamentous tissue in the inner ear leads to conductive hearing loss. There may be fibrotic changes in the myocardium, pericardium, pleura, or lung. Glomerulonephritis with renal crisis is a frequent cause of morbidity and mortality. Mucosal gastrointestinal lesions occur, preferentially in the distal esophagus. Keratoconjunctivitis, lens subluxation, and cataracts are typical ocular signs, and musculoskeletal changes, including atrophy and osteolytic lesions, joint symptoms, and myopathy occur in...

Rehabilitation Outcome

Surgical cure rates are hard to define because of the small numbers of reported cases. Progression-free 3-year survival rates of less than 10 are reported. Local radiation therapy or focused beam irradiation have been used, resulting in a 5-year survival rate of less than 50 . In contrast, recent results with proton beam irradiation in children show a disease-free survival rate of 68 at 5 years. Of the remaining living patients from our series treated with proton beam therapy, one has had a recurrence, which may be related to inadequate surgical decompression of the nervous tissue and thus limitation of radiation dosage. To improve the results, close cooperation is necessary between the radiation therapist and the surgical team. Complications include pituitary dysfunction, sinusitis, hearing loss, radiation necrosis, and possibly, second tumors.

Box 291 Useful Internet sites for information regarding MR safety

These.2 Despite this, MRI is extremely noisy. The noise occurs during the rapid alterations of the magnetic currents within the gradient coils. These currents, in the presence of the static magnetic field, produce significant forces on the gradient coils, so causing them to impact upon their mountings.11 The noise level is independent of the strength of the static magnetic field.17 Temporary hearing loss has been reported following MRI,18 although there are no substantiated reports of permanent loss.

Congenital Syndromesdiseases

Patients with this syndrome have Lentigenes (1- to 5-mm brown macules on back, thorax, and neck), Electrocardiographic conduction defects, Ocular hypertelorism, Pulmonic stenosis (and other cardiovascular system abnormalities such as hypertrophic cardiomyopathy), Abnormalities of genitalia (hyopgonadism), Retardation of growth, and Deafness of sensorineural origin (30) (Fig. 2). Patients with LEOPARD syndrome are predisposed to sudden death ifthere is coexistent hypertrophic cardiomyopathy (31).

Creativity and the Transforming Illness

Nietzsche's phrase What does not destroy me, makes me stronger is evident in the transformational power of major illness. Throughout the centuries, visual artists of diverse backgrounds have used creativity to turn poor physical health into a transforming illness, an experience that alters both their lives and their work. There are as many types of transforming illnesses as there are people who have them. Artists have used conditions such as cancer, AIDS, arthritis, quadriplegia, learning disorders, vision problems, deafness, and the aftermath of serious accidents as opportunities for self-evolution. A transforming illness may be an acute episode or a chronic condition, yet whenever it appears, life takes a new trajectory and things are never the same.

The Geriatric History

If there is a hearing impairment, sit facing the patient, as close as possible and at ear level with the patient. Make sure that the patient is wearing, if required, the hearing aid or other assistive device. Try to minimize both audible and visual distractions. Speak in a slow, low-pitched, and moderately loud voice. Allow the patient to observe your lips as you talk. Finally, confirm with the patient that he or she is being understood by repeating portions of the history. Hearing impairment Incontinence (fecal and urinary) Infections (recurrent)

Other Chemotherapeutic Agents

Direct toxicity of the drug on the organ of Corti appears to be the cause of the induced deafness, because nystagmus and vertigo have rarely been reported. Deafness is more common in elderly patients and is exacerbated by radiation therapy to the head. The patient cannot hear high-frequency sounds the hearing loss is bilateral and largely reversible, but it can be permanent. Deafness often begins within 3 to 4 days of the initial treatment and slowly improves over the succeeding weeks after treatment is stopped. The combination of slow infusion and pretreatment with intravenous hydration and often mannitol to enhance renal excretion has reduced the problem of ototoxicity. y , y , y

Neurofibromatosis Type

Diagnostic criteria for NF2 devised at the 1987 NIH Consensus Development Conference on Neurofibromatosis37 were subsequently revised by the National Neurofibromatosis Foundation (NNFF) Clinical Care Advisory Board (Table 108-3). Patients with NF2 clinically may report a variety of difficulties. The majority of these are neurologic, including hearing impairment, tinnitus, imbalance, weakness, seizures, numbness or paresthesias, or impaired vision. Approximately 11 are identified while asymptomatic as a result of screening within affected families. Only 10 of patients become symptomatic before age 10.15 In addition, new signs and symptoms may develop with increasing age, and therefore the evaluation of an NF2 patient must involve long-term, continued serial assessments. ticular importance, given the fact that this group of patients is also at risk for bilateral hearing loss. Additional ocular manifestations of NF2 include retinal hamartomas, epiretinal membranes, and rarely Lisch...

Benign Neonatal Familial and Nonfamilial Convulsions Benign idiopathic neonatal convulsions BINNC

Retardation, and a characteristic EEG pattern known as hypsarrhythmia. The syndrome is named for WJ West, the nineteenth century neurologist who described the features he observed in his own son. Infantile spasms occur in 24 to 42 of 100,000 births. y Spasms and psychomotor retardation appear in the first year of life in 85 percent of cases, the majority between 3 and 7 months. Infantile spasms are sudden, brief, usually bilaterally symmetrical flexor contractions of the neck, trunk, or limbs. Eye movements, autonomic signs, and brief lapses of consciousness may be observed. Less commonly, extension of the head, trunk, and limbs may be seen. Spasms are commonly repetitive, occurring in clusters on awakening, or during drowsiness, handling, feeding, or fever. Initially, the movements can be so slight that they may go unnoticed. Neurodevelopmental abnormalities may precede the onset of spasms. Moderate to severe cognitive disability occurs in 76 to 95 percent of patients. Associated...

Varicella Zoster Virus

Both herpes simplex virus and VZV are etiological agents of Bell's palsy. VZV is more likely than HSV to cause a complete seventh nerve paralysis with concomitant severe pain and sensorineural hearing loss. Complete recovery of facial function may not occur. '1211

The anterior inferior cerebellar artery AICA

The classic AICA syndrome includes vertigo with vomiting and nystagmus (vestibular nuclei, vestibular nerve or labyrinthine artery), ipsilateral deafness with tinnitus (cochlear nerve or cochlear artery), ipsilateral peripheral-type facial palsy (facial nucleus or fascicle of VII), ipsilateral facial hypesthesia (trigeminal nuclei or fascicle), ipsilateral Horner's syndrome (descending sympathetic tract), ipsilateral ataxia, dysar-thria (middle cerebellar peduncle and cerebellum) and contralateral thermoalgesic sensory deficit (spi-nothalamic tract). It is frequently misdiagnosed as Wallenberg syndrome, but the main clinical distinctions are the hearing loss and the peripheral-type facial palsy. Occasionally, horizontal ipsilateral gaze palsy or dysphagia are also present. More rarely, AICA territory stroke can present as an isolated vertigo or isolated cerebellar syndrome.

Psychotic disorders hallucinations and delusions

Hallucinations in stroke patients are predominantly visual and can be due to (1) sensory deprivation poor vision (Charles Bonnet syndrome), darkness, deafness . . . (2) delirium and substance withdrawal (alcohol, drugs) (3) rostral brainstem and thalamic lesions (peduncular hallucinosis) (subcortical hallucinations) (4) partial occipital lesions (release hallucinations) (cortical hallucinations). Functional imagery studies showed that in subjects with visual hallucinations there was activation of the ventral extrastriate visual cortex and that the type of hallucinations reflected the functional specialization of the activated region.