Help For Hearing Loss Sufferers

Hearing Aids Inside Out

Hearing Aids Inside Out

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

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Breaking The Sound Barriers

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

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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...

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...

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....

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...

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).

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...

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...

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...

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-

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.

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.

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 Management Goals

Such as Meniere's disease, treatment directed at the cause may be used, but there is no effective pharmacological management of tinnitus or hearing loss. Hearing aids are indicated when hearing loss is significant and aidable, meaning that improvement in word recognition scores may be obtained with amplification. Hearing aids are expensive. They are often not covered by standard insurance policies, and there are considerable performance differences between basic and deluxe models. For these reasons, hearing aids should ideally be fit by an experienced audiologist who offers at least a 1-month tryout period.

Clinical Presentation

Initial symptoms, such as vertigo, dizziness, and tinnitus, result from irritation of the vestibular or the cochlear part of the eighth nerve. At the beginning these symptoms may be slight and temporary with several relapses occurring over weeks or months, or they may develop so slowly that they are neglected for a long time. Vestibular symptoms may be noticed by patients as a short intermittent feeling of being drawn to one side and almost losing their balance mostly these symptoms are attributed to blood pressure disturbances. Acoustic symptoms include high-pitched (rarely low-pitched) tinnitus and decreasing hearing. Hearing loss may present as an acute attack with or without recovery or as a progression over a couple of years. Some patients become aware of their hearing problem only when using the phone on that ear. Then an otorhinolaryngologist is contacted.

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.

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...

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...

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...

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...

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.

Inspect the Tympanic Membrane

Normal Tympanic Membrane Labeled

Healthy tympanic membranes are usually pearly gray. Diseased tympanic membranes may be dull and become red or yellow. Is the eardrum injected Injection refers to the dilatation of blood vessels, making them more apparent. The blood vessels should be visible only around the perimeter of the membrane. Dense, white plaques on the tympanic membrane may be caused by tympanosclerosis, which is caused by deposition of hyaline material and calcification within the layers of the tympanic membrane. This condition is commonly (in 50 to 60 of cases) secondary to the insertion of ventilation tubes. The classic horseshoe shape of tympanoscle-rosis is seen in the tympanic membrane shown in Figure 11-18. Despite the size of these lesions, they usually do not impair hearing and are rarely of clinical importance. If the lesion extends into the middle ear, however, conductive deafness may result. onto the ossicles of the middle ear. The ossicles may become eroded, with the development of a conductive...

The Written Physical Examination

Left Lower Abdominal Tenderness

Henry is a 65-year-old man in no acute distress. Physical examination reveals systolic hypertension, retinal changes suggestive of sustained hypertension, a mild cataract in his right eye, a conductive hearing loss in his right ear, tonsillopharyngitis, and gynecomastia. Cardiac examination reveals aortic insufficiency. Peripheral vascular examination reveals possible atherosclerotic disease of the right carotid artery and mild venous disease of the lower extremities. The patient has a right, easily reducible inguinal hernia. A left-sided varicocele is present. Mild osteoarthritis of the hands is also present.

Mecanism Of Centripetal Lipid Accumulation Supraclavicular Fat And Face

Benign lesions of the lateral skull base and cerebellopontine angle (CPA) often produce neurotologic symptoms such as hearing loss, dizziness, imbalance, facial weakness, and facial hypesthesia. Clinicians who manage patients with skull base lesions should have a working understanding of the clinical evaluation of these symptoms. This chapter focuses on the neurotologic evaluation of patients with benign lesions of the lateral skull base and CPA. We begin with a brief description of the more common surgical approaches to these regions from the neurotologic perspective. We then focus on key elements of the neurotologic examination and clinical testing that assist in establishing the diagnosis and selecting the most appropriate clinical management.

Clinical clues to differentiate posterior from anterior circulation strokes

Motor, cerebellar and sensitive signs are less specific in brainstem lesions, but the presence of bilateral or crossed signs is suggestive. The former is due to the bilateral supply of the brainstem by one midline artery (the BA). The latter is caused by ischemia of cranial nerves and fascicles that produce ipsilateral signs and simultaneous damage to the long sensory and motor tracts that cross in the caudal parts of the brainstem. Truncular ataxia is quite characteristic of brainstem lesions, and acute unilateral deafness (with or without vertigo) suggests ischemia in the AICA territory. Clinical symptoms and signs that point to a posterior circulation stroke preceding TIAs and strokes in the days and hours before the infarct, headache, typically ipsilateral to the infarct, a disconjugate gaze or a conjugate gaze paresis with the eyes deviated toward the hemiparesis (brainstem lesion), a vertical gaze paresis (dorsal mesencepha-lic lesion), nystagmus, ocular tilt reaction (triad of...

Chronic Progressive External Ophthalmoplegia and Kearns Sayre Syndrome

And hearing loss cardiac conduction defects, arrhythmias, and dilated or hypertrophic cardiomyopathies CNS dysfunction, including dementia, seizures, and ataxias peripheral nervous system damage, including motor sensory neuropathies mitochondrial myopathy and mutisystemic organ involvement in the form of diabetes mellitus, hypoparathyroidism, glomerulosclerosis, proximal renal tubule dysfunction, gastrointestinal motility problems, and respiratory failure.

Basic Principles of Geriatric Medicine

A third principle is the underreporting of illness. When an interviewer asks a geriatric patient about various symptoms, the patient may fail to report blindness caused by a cataract, deafness caused by otosclerosis, pain in the legs at night, urinary incontinence, constipation, confusion, and so forth. The geriatric patient may believe that these symptoms are normal for a 75- or 80-year-old person. Abdominal pain and other gastrointestinal complaints such as increased gas are commonly mistaken by geriatric patients as a normal part of aging. Sometimes a patient may say, ''Nothing can be done about it, so I don't want to bother anyone by mentioning it.''

Syndromes Primarily Involving Hearing

Hearing loss is highly prevalent, especially in the older population, and three types are commonly encountered conductive, sensorineural, and central hearing loss. In conductive hearing loss, sound is not transmitted into the inner ear. Diagnosis is ordinarily made via observation of an air-bone gap on audiometry, meaning that hearing is superior when sound is transmitted in such a way that it bypasses the middle ear ossicular chain. Causes include a buildup of ear wax, foreign body in the ear canal, otosclerosis, external or middle ear infections, allergy with serous otitis, and perforation of the tympanic membrane. Characteristically, hearing aids work well for this population. Sensorineural hearing loss is the most common type of hearing loss, occurring in 23 percent of the population older than 65 years of age. The term sensorineural is used to indicate that there is either a cochlear or an eighth nerve lesion. The diagnosis of a sensorineural pattern hearing loss is made through...

Neurosurgical conditions

Limit (in anterior fossa fractures), or bruising over the mastoid process (Battle's sign) in fractures of the petrous bone. Petrous fractures can damage the middle or inner ear and may have associated bleeding from the external meatus, deafness, dizziness or facial nerve palsy. If the dura underlying a base of skull fracture is torn, there may be a CSF leak through the nose or the ear, or air many enter the subarachnoid space (pneumocephalus). In either event there is a risk of bacterial meningitis. If the dural tear does not heal within 1-2 weeks, surgical repair is usually indicated. Recently published guidelines do not recommend prophylactic antibiotics for a CSF leak.

Schwannomas of the Jugular Foramen Nerves

The most common symptom reported was hearing loss. Besides paresis of the nerves, patients can have vagoglos-sopharyngeal pain. Dysphagia, dysphonia, and persistent toothache have been reported. Schwannomas of the jugular foramen may mimic the clinical signs of glomus jugulare tumors in every aspect. Keye classified these tumors into three groups (1) type A is confined to the intracranial compartment (2) type B invades mainly the bone and (3) type C is primarily extracranial.7

Table 556 Peripheral Neurotoxicity Of Antibiotics

The toxicity of all aminoglycosides is similar--mainly ototoxicity and neuromuscular blockade (llTable.lll55z7.). Acute ototoxicity is based on calcium antagonism and blockade of ion channels, and chronic mechanisms are related to tissue-specific toxicity of a noxious metabolite. The frequency of ototoxicity ranges from 2 to 4 percent in retrospective studies and up to 25 percent in studies with specialized testing. The incidence and severity of damage appear to increase with patient age, total drug dose, and concomitant use of other ototoxic drugs. y Auditory toxicity is more common with the use of amikacin and kanamycin, whereas vestibular toxicity predominates following gentamicin and streptomycin therapy. Tobramycin is associated equally with vestibular and auditory damage. Cochlear toxicity is more often silent, as the hearing loss first affects the high frequencies (greater than 4000 Hz) before involving the speech frequencies. Cochlear toxicity presents clinically as deafness,...

Vertebrobasilar Stroke Syndromes

The AICA syndrome causes a ventral cerebellar infarction that has a characteristic clinical picture y , y (see TabJe 2.2-2 ). The signs and symptoms that are seen include vertigo, nausea, vomiting, and nystagmus due to involvement of the vestibular nuclei. There may be ipsilateral facial hypalgesia and thermoanesthesia and corneal hypesthesia due to involvement of the trigeminal spinal nucleus and tract. There is ipsilateral deafness and facial paralysis due to involvement of the lateral pontomedullary tegmentum. Ipsilateral Horner's syndrome is present due to compromise of the descending oculosympathetic fibers. There is contralateral trunk and extremity hypalgesia and thermoanesthesia due to involvement of the lateral spinothalamic tract. Finally, there is ipsilateral ataxia and asynergia due to involvement of the cerebellar peduncle and cerebellum. peduncle. Choreiform dyskinesias may be present ipsilaterally. Contralaterally, there is hearing loss due to lateral lemniscus...

Hereditary causes of stroke single gene disorders and their clinical presentation

Blood lactate levels are elevated, indicating dysfunction of the respiratory chain. Most commonly, MELAS is associated with a mitochondrial DNA point mutation at position 3243 within the tRNA encoding gene. Many different phenotypes, alone or in combinations, have been reported with this mutation (hearing impairment, cognitive decline, progressive external ophthalmoplegia, or epilepsy).

Inspect the Hard and Soft Palates

Petechiae Hard Palate

Is the palate intact Figure 12-32 shows a severe cleft palate. Clefts of the palate and lips are distinct entities but are closely related embryologically, functionally, and genetically. The incidence of an isolated cleft palate is 1 per 1000 births. Clefts of the palate vary widely in size and shape. They can extend from the soft palate, to the hard palate, and to the incisive foramen. Recurrent otitis media, hearing loss, and speech defects are frequent complications.

Directed Neurological Examination

The examination proceeds with an inspection of the external ear and ear canal looking for malformations, infections, masses, or asymmetry. Next, the tympanic membranes should be inspected for wax, perforation, otitis, or mass lesions. It is usually prudent to remove wax before embarking on more sophisticated diagnostic procedures. The tympanic membranes contribute about 20 db to the hearing level. Disorders such as perforation, scarring, fluid accumulation, or wax impaction can cause a conductive hearing loss. A normal tympanic membrane is translucent. Fluid behind the tympanic membrane imparts a straw color. For bedside hearing assessment, the Rinne's and Weber's tests can be used. In the Weber's test, a vibrating 512-Hz tuning fork is placed on the patient's forehead. If the sound predominates in one ear, the patient may have either a conductive hearing loss in that ear or a contralateral sensorineural hearing loss. In the Rinne's test, the base of a vibrating tuning fork is placed...

Posttraumatic Epilepsy

Labyrinthine concussion without a skull fracture is the most common site. Conductive hearing loss follows longitudinal temporal bone fractures in over 50 of cases. Transverse fractures result in vestibular and cochlear nerve laceration in over 80 Findings associated with a fracture of the petrous portion of the temporal bone include hemotympanum or tympanic membrane perforation with blood in the external canal, hearing loss, vestibular dysfunction, peripheral facial nerve palsy, CSF otorrhea, and ecchymosis of the scalp over the mastoid bone (Battle's sign). Benign positional vertigo occurs in about 25 of patients following head trauma Examine the external canals and tympanic membranes. An audiogram including pure-tone and speech audiometry, acoustic reflexes, and middle ear function is used to assess hearing loss. An electronystagmogram (ENG) is used to assess vestibular function. CT scan to evaluate temporal bone trauma A hearing aid may help those with sensorineural hearing loss....

Structure and Physiology

Senile Loss Elasticity

Degeneration of the organ of Corti may result in presbycusis, an impaired sensitivity to high-frequency tones. Patients experience a slowly progressive hearing loss with a consistent pattern of pure tone loss. Otosclerosis may produce conductive deafness, as does the excessive cerumen accumulation so commonly seen in older individuals. A degeneration of the hair cells in the semicircular canals may produce vertigo.

Patent Ductus Arteriosus

First trimester maternal rubella with rash carries an 80 incidence of intrauterine viral infection139 and results in deafness and cataracts (Fig. 20-10) and congenital malformations of the heart in two thirds of offspring. Patent ductus arteriosus accounts for one third of these malforma-tions79 and is characterized by maturational arrest and an immature wall of the type found at 16 weeks' gestation (see earlier).79 should be examined for cataracts, deafness, and mental retardation (Fig. 20-10).118 Another distinctive phenotype is the overlapping fingers (clinodactyly), rocker bottom feet, and lax skin of trisomy 18 (Fig. 20-11).29,124,168 Char syndrome is an inherited disorder that maps to chromosome 6p12-p21 and is characterized by patent ductus arteriosus, facial dys-morphism, and abnormalities of the hand.158,162,170

Clinicopathologic Correlations

Acute Otitis Externa

Acute otitis externa is a common inflammatory condition of the external ear canal, most often caused by Pseudomonas aeruginosa. The prominent symptom is severe ear pain (otalgia) accentuated by manipulation of the pinna and especially by pressure on the tragus. Edema of the external ear canal, erythema, and a yellowish-green discharge are prominent signs of this disease. Commonly, the canal is so tender and swollen that adequate visualization of the entire canal and tympanic membrane is impossible. ''Swimmer's ear'' is a form of otitis externa in which there is a loss of the protective cerumen, and chronic irritation and maceration by water and bacteria occur. Itching is a common precursor of otalgia. Figure 11-26 shows the external ear canal of a patient with acute otitis externa. Notice the follicular appearance of the canal, which resulted from epithelial swelling. As the condition progresses, the lumen may be occluded, producing conductive deafness. Acute otitis media is a...

Facial Nerve Paralysis

If the facial weakness is secondary to Ramsay Hunt syndrome, treatment is similar to that of Bell's palsy, but the clinical course and expected outcomes differ. This syndrome is caused by herpes zoster (rather than herpes simplex) involvement of the facial (geniculate), vestibulocochlear, and or trigeminal ganglia. The infection causes pain and eventually vesicular eruptions around the auricle and external ear canal. Vesicles may appear only in the pharynx or hard palate in some cases. Facial weakness and at times dense paralysis are common. Hearing loss, tinnitus, and persistent vertigo also occur in 20 to 30 of patients (Adour, 1994). As with Bell's palsy, prompt initiation of oral steroids and acyclovir should begin when the diagnosis is suspected. This therapy can lessen vertigo and improve recovery of facial Ramsay Hunt syndrome with cranial nerve VII involvement, vestibular(vertigo), cochlear (hearing loss) nerve function, although outcomes are not as favorable as for Bell's...

Other Disorders of the Skull and Spine Dwarfism

Congenital fusion of the cervical vertebrae is known as the Klippel-Feil anomaly. Presumably this reflects a failure of segmentation rather than a secondary fusion. This anomaly can be part of a number of syndromes (such as Turner's, Noonan's, and Wildervanck's syndromes) but can also occur sporadically or be inherited in isolation as an autosomal dominant or autosomal recessive trait. Vertebral fusions can extend beyond the cervical spine, but most commonly they are restricted to levels C2 and C3 or C5 and C6. Clinically, there is a short neck, low posterior hairline, and limitation of neck movements, particularly rotation and bending to the sides. Atlanto-occipital anomalies are frequently associated, and one of the major reasons for associated morbidity. Kyphosis and scoliosis are often associated, as is spina bifida occulta in some cases. y Many patients also have a variety of genitourinary anomalies, including unilateral renal agenesis. '92' Hearing impairment can be noted in...

What if my testosterone level is low What are the risks and benefits of testosterone therapy

HA in 16 , flushing in 10 , dyspepsia in 7 , visual disturbance in 3 , priapism uncommon. NAION (nonarteritic anterior ischemic optic neuropathy) and hearing loss have been reported in individuals taking PDE 5 inhibitors, but a causal relationship has not been identified. The risk factors for NAION are similar to those for ED, such as age > 50 yr, HT, increased cholesterol, and DM. Hearing loss has also been reported in patients taking PDE-5 inhibitors. As with NAION, a causal relationship has not been established. Hearing loss see sildenafil side effects.

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...

Functional anatomy

Deafness in one ear, although this is rare due to significant cross midline communication in the brainstem. Auditory symptoms are associated with brainstem strokes and may include hearing loss (if bilateral stroke), phantom auditory perceptions (tinnitus hallucinations) and increased sensitivity to sound (Hausler and Levine, 2000). Lesions to the auditory cortex in the temporal lobe (cortical deafness) may mean that although the patient is unable to consciously hear sound, they may be able to reflexively respond to sound thus 'appear' to hear Lesions to adjacent association areas (Wernicke's area) result in patients being unable to comprehend the meaning of sounds or words, known as receptive aphasia or agnosias to particular sounds.

Acute Bacterial Meningitis

Cranial nerve palsies, and most notably sensorineural hearing loss, are a common complication of bacterial meningitis and may be present early in the course of the illness. A stiff neck is the pathognomonic sign of meningeal irritation, resulting from a purulent exudate or hemorrhage in the subarachnoid space. Nuchal rigidity or meningismus is present when the neck resists passive flexion. Kernig's sign is also a classic sign of meningeal irritation, and as originally described by Kernig, it requires the patient to be in the seated position. Kernig noted that attempts to passively extend the knee while the patient was seated were met with resistance in the presence of meningitis so that a contraction of the extremities was maintained. y , y Jozef Brudzinski described at least five different meningeal signs. His best known sign, the nape of the neck sign, is elicited with the patient in the supine position and is positive when passive flexion of the neck results in spontaneous flexion...

Posterior Fossa Anomalies

In contrast to these cerebellar syndromes, the brain stem can also be the primary area affected. In its broadest definition, Mobius' syndrome refers to a combination of bilateral symmetrical and asymmetrical facial weakness associated with abnormalities of horizontal gaze, most often deficiencies of abduction. Other cranial nerve functions can be impaired as well, especially those of cranial nerves IX, XII, V, and occasionally, III. The impairment of lower cranial nerves can lead to early feeding difficulties, problems with swallowing, and atrophy of the tongue that is frequently unilateral. Neurosensory deafness may also be associated. A variety of somatic anomalies may be present in patients with Mobius' syndrome.y At least 50 percent of patients have limb anomalies. Thirty percent have talipes, and 20 percent exhibit hypoplasia of digits, transverse terminal defects, or syndactyly. The Poland anomaly and the Klippel-Feil anomaly can also be associated with the condition. Concurrent...

Physical Appearance

The rubella syndrome is characterized by cataracts, retinopathy, deafness, hypotonia, dermatoglyphic abnormalities, and mental retardation.37 Height and weight are usually normal for age despite intrauterine growth retardation. Patent ductus arteriosis (see Chapter 20) and stenosis of the pulmonary artery and its branches are the most frequent types of coexisting congenital heart disease.37

Autosomal Recessive Inheritance

Retinal pigmentary degeneration, polyneuropathy, and ataxia sensorineural deafness, anosmia, and cardiomyopathy are usually present abnormalities of the eyes, skin (icthyosis), and bone are often present lcthyosis and hypogonadism are major features microcephaly, sensorineural deafness, polyneuropathy, and hypoplastic teeth and nails are less frequent

Language Related Disorders

PURE WORD DEAFNESS Pure word deafness is a rare prelanguage syndrome in which only reception of spoken language is involved. Individuals with pure word deafness do not understand spoken language and cannot repeat. However, they are able to hear and identify adequately nonverbal sounds such as a whistle, a telephone ring, and a dog bark. Their reading is intact, and they often carry with them a writing tablet for others to use. y Some cases of pure word deafness have associated paraphasic output and mild written language comprehension deficits. These cases should be classified as Wernicke's aphasias with predominant word deafness and relatively less word blindness (reading comprehension). Pure word deafness is not a disturbance of language ability. Delivery of the auditory signal to the area mediating language interpretation is defective, which is a sensory transmission problem rather than a language disturbance. Pure word deafness resembles deafness more than aphasia. It has also been...

Neuromuscular Junction And Muscle

And neuronal loss within the temporal lobes initially manifests as anxiety and depression, then progresses to loss of recent memory, agitation, confusion, hallucinations, behavioral abnormalities, hypersomnia, generalized or partial complex seizures, and progressive dementia. y Similarly, any level of the brain stem may be impaired with signs and symptoms reflective of that level of involvement. Involvement of the medulla oblongata produces nausea, vomiting, vertigo, nystagmus, ataxia, and bulbar palsy. Less commonly, disorders of eye movements occur when the midbrain or pons is involved. Extrapyramidal syndromes including parkinsonism, dystonia, respiratory abnormalities, deafness, and myoclonus of the bronchial musculature have been observed. y

Extracranial Granulomatous Arteritis Giant Cell Temporal Arteritis

Vertigo or unilateral hearing loss may rarely be the result of an infarct of the vestibulocochlear nerve. Other cranial nerves, such as the fifth, seventh, or twelfth, have been affected in isolation or as part of a mononeuritis multiplex. Occasionally, other cranial neuropathies have occurred causing facial pain, hemianesthesia of the tongue or lingual paralysis.

Autosomal Dominant Cerebellar Ataxia

Clinical Features and Associated Disorders. FRDA usually appears before the age of 25 years, most often in children between 10 and 15 years old. In most patients, ataxia of gait and stance is the first manifestation of the disease. In others, skeletal deformities, in particular scoliosis, are present prior to the onset of ataxia. A number of clinical symptoms are present from the beginning of the disease in virtually all patients. These include progressive ataxia, areflexia of the lower limbs, and impaired vibration or position sense. About two thirds of the patients have extensor plantar responses. There is progressive weakness of the extremities, which is due to pyramidal tract dysfunction and distal muscle atrophy. Despite pyramidal involvement, muscle tone is usually normal or decreased. However, some patients may suffer from spontaneous flexor spasms. Less than 10 percent of patients with FRDA experience a reduced appreciation of pain and light touch sensation. Oculomotor...

Vertebrobasilar System

Ipsilateral deafness, ipsilateral facial weakness, decreased sensation, ipsilateral face Nystagmus, ipsilateral deafness, diplopia (CN IV) Acute hearing loss can occur with AICA infarctions. Vascular lesions of the auditory cortex do not lead to complete deafness, even if the lesions are bilateral. Pure word deafness can occur with unilateral or bilateral temporal lesions affecting Heschl's gyri. Due to the binaural representation of the ascending auditory tracts above the level of the cochlear nuclei, lesions of the brain stem involving the auditory pathways do not cause deafness.

Sex Chromosomal Abnormalities

Turner's syndrome, a form of gonadal dysgenesis resulting from a 45,X karyotype (X-chromosomal monosomy), is characterized by female phenotype, short stature, a shieldlike chest, a short and sometimes webbed neck, low- set ears, high-arched palate, small mandible, and sexual infantilism. y The frequency of 45,X in female live births is 0.1 to 0.6 per 1000. A variety of other malformations can be associated, including congenital lymphedema, particularly of the hands and feet, cardiac and renal defects, skeletal anomalies, and abnormalities of the nails. An increased number of pigmented nevi has also been reported. Other disorders have been associated with this disorder, including Hashimoto's thyroiditis, obesity, inflammatory bowel disease, and rheumatoid arthritis. Nerve deafness occurs in approximately half the patients, and olfactory as well as taste deficits have been described. Eighteen percent of patients studied in one series were mentally retarded, although this high prevalence...

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...

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.

Associated Neurological Findings

The history of previous subacute monocular visual loss suggests a diagnosis of multiple sclerosis. Some degenerative ataxias are associated with gradual visual loss due to retinitis pigmentosa. Diplopia, facial numbness, facial droop, vertigo, or hearing loss resulting from associated cranial neuropathies (cranial nerves III, IV, V, VI, VII, and VIII) associated with ataxia suggest a disorder in the brain stem. Tumors of the cerebellopontine angle, such as acoustical neurinomas, are frequently associated with nerve palsies of cranial nerves V, VII, and VIII.

Clinical Manifestations and Pathology

In congenitally acquired rubella, the fetal infection may result in abortion, stillbirth, congenital malformations, or growth retardation. The congenital rubella syndrome is the result of inhibition of cell multiplication in the developing fetus and a chronic infective state that may persist for many months after birth. Some consequences of fetal infection may not become apparent until years after birth. Common congenital abnormalities and active infective processes at birth include cataracts, deafness, central nervous system defects leading to mental retardation, structural defects of the heart and myocarditis, bone lesions, pneumonitis, and hepatitis.

Emergency Department Treatment and Disposition

Initial evaluation is directed by the history. The examination should include a thorough examination of the ear (including sensorineural or conductive hearing loss), the eye (including lacrimation), and the cranial nerves especially extraocular muscles (EOMs). Motor function of the seventh cranial nerve is evaluated by having the patient raise their eyebrows, smile, pucker, and frown. No single laboratory test is diagnostic. Screening CT or MRI of the head is of little value in the absence of additional findings on physical examination.

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

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)

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.

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).

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.

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.

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...

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

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.

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,...

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.

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.

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

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...

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...

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,...

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.

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.

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).

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.

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.

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.

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).

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.

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).

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,...

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...

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.

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

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...

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

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.

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