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29.10.2013
Involvment of the autonomic system manifests as intermittent fever, hypohidrosis, and poor temperature control.  Some patients have periodic crises of severe pain in the extremities as well as intermittent epigastric pain. GeneticsThis is an X-linked disorder and generally assumed to be recessive although some have suggested dominance since most heterozygous females have significant manifestations that can be life-threatening.  The mutations in the responsible gene (GLA), located at Xq22, involve a variety of deletions, rearrangements and single base pair changes. The milder disease and increase in variable expressivity among females is likely a reflection of variable patterns of X-inactivation. Increased tortuosity of retinal arterioles is also seen in Williams syndrome (194050), and in a condition known as retinal arteriolar tortuosity (611773, 180000). Continuous release of cardiac troponin I (cTNI) with elevated serum levels may be a clue to the severity of heart involvement. Juan P, Hernan A, Beatriz SA, Gustavo C, Antonio M, Eduardo T, Raul D, Margarita L, Mariana B, Daniela G, Marina S. Beck M, Ricci R, Widmer U, Dehout F, de Lorenzo AG, Kampmann C, Linhart A,Sunder-Plassmann G, Houge G, Ramaswami U, Gal A, Mehta A.
MIXED HEARING LOSS Whenever the hearing loss of a patient includes a mixture of both conductive and sensorineural characteristics, is said to have a mixed hearing loss. Superior ventral cochlear nucleus (SVCN) Erythroblastosis typically causes hearing loss that centers around 3000–4000 Hz. Central Auditory Processing Disorders (CAPD) CAPD characterized by decreased understanding of speech in the presence of background noise. FUNCTIONAL HEARING LOSS Functional or psychogenic hearing loss is the customary diagnosis when there is no organic basis for the patients apparent deafness. Functional Overlay It is entirely possible for hearing loss of functional origin to be superimposed on true organic hearing impairment. History: A 34-year-old woman with otosclerosis and hearing loss for over 10 years Refuse to use hearing aids She often says, What? Audiometric Patterns in Functional Hearing Loss There is no characteristic audiometric pattern in functional hearing loss, but the consistent inconsistencies serve to alert the physician. Malingering Malingering is the deliberate fabrication of symptoms that the patient knows do not exist. Damon Cawley a Physical Therapist who is currently treating patients at Natural Healing Arts Medical Center in both Bradenton and Sarasota Florida joins eHealth Radio and the Health Care & Pain Management Channels. Tell me a little about Natural Healing Arts Medical Center and how this clinic differs from other offices that offer Physical Therapy? You mentioned earlier that each clinician has their own special tools in their toolbox of treatments.
Chronic kidney disease and an uncertain diagnosis of Fabry disease: Approach to a correct diagnosis. Fabry Disease: Multidisciplinary Evaluation After 10 Years of Treatment with Agalsidase Beta. Life expectancy and cause of death in males and females with Fabry disease: findings from the Fabry Registry. Ophthalmological manifestations of Fabry disease: a survey of patients at the Royal Melbourne Fabry Disease Treatment Centre. Trussell the CEO of Webstar Behavioral Health and author of How Families Flourish and Kicking Depression Out of Your Life joins eHealth Radio and the Mental Health Channel to talk about how to overcome the holiday blues.
Elmore Alexander, Founder and CEO of SmartPlex Atlanta that specializes in hair regrowth and restoration, skin rejuvenation and sexual restoration joins eHealth Radio and the Female Health & Sexual Health Channels. Elmore Alexander is a Board Certified Osteopathic Physician with an extensive background in holistic medicine, Sports Medicine, and Rejuvenation Aesthetics.
Alexander has performed graduate level, biochemical research and is a formulator of topical health related products.
Alexander has also has served as the COO of the Primary Care Division of Coastal Carolina Neuropsychiatric Center in Jacksonville, NC.


Hearing deficiency may have started originally as a conductive failure (otosclerosis) and later developed a superimposed sensorineural component. In clinical practice, most cases with an original sensorineural etiology remain in that classification without an added conductive element. In chronic otitis media some toxic inflammatory metabolite produces a cochleitis or labyrinthitis. This example a warning to physicians to avoid assuring any patient that his hearing loss can be corrected merely by removing cerumen, because mixed hearing loss may be found subsequently.
In every case of mixed hearing loss one should determine how much of the deficit is conductive and how much is sensorineural. If the patient does not understand any better with a hearing aid or when the voice is raised, hearing is not improved even if the conductive portion of the mixed hearing loss is corrected. 17 The auditory pathway consists of a series of transducers that repeatedly change the speech stimulus so that it can be handled effectively by the cortex.
Characteristic Features of central hearing loss : Hearing tests do not indicate peripheral hearing impairment.
This may be caused by injury to SVCN spheroid cells, the second-order neurons of the ascending auditory pathway, even in the presence of normal hair cells in the organ of Corti.
Common complaints include inability to study or read in the presence of noise, slowing of reading speed caused by noise(vacuum cleaners or air conditioners ) and suspicion by family members that the patient has a hearing loss.
The inability to hear results entirely or mainly from psychological or emotional factors, and the peripheral hearing mechanism may be essentially normal. Psychogenically induced hearing loss usually is a uniform flat- tone loss in all frequencies, suggestive of a well-marked conductive impairment. Usually, the hearing impairment is bilateral, and the bone conduction level is the same as the air conduction level. The risk of ischemic strokes is increased.  Cardiac manifestations include hypertrophic cardiomyopathy (60%), mainly involving the left ventricle, and dysfunction of the mitral and aortic valves (10 to 25%). As a licensed professional counselor and positive psychology behavioral coach, he has dedicated his career to helping people eradicate mental health problems from their lives. The difficulty may have been sensorineural in the beginning ( presbycusis ), and a conductive defect (middle-ear infection ) may have developed subsequently. In contrast, most cases that start as conductive hearing impairment later develop some sensorineural involvement.
The sensorineural deficit may be caused by penetration of the oval window with exposure of the perilymph. Prognosis: In mixed hearing loss the prognosis depends on the relative proportion of conductive and sensorineural pathology. There is no characteristic audiometric pattern, except that the disparity between the hearing level and the speech interpretation is quite marked. Central pathology must be included in the differential diagnosis of the hearing loss producing a 4000-cycle dip on the audiogram.
If people talk to a person with CAPD while he or she is involved in auditory concentration such as listening to a television program, the person will often not hear. The basis for functional hearing loss in most patients is psychogenic, the product of emotional conflict, ( neurotic anxiety ). Hysterical blindness and paralysis are other examples of the same type of somatization or conversion reaction. In a patient with unilateral functional deafness there may be complete absence of bone conduction on the side of the bad ear, though the good ear has normal acuity.
If the patient claims he has one good and one bad ear, and the examiner obstructs his good ear with a finger, then shouts into it loudly enough to be heard easily by bone conduction alone, the malingerer claims he hears nothing. Alexander served the boards and financial committees of Georgia Blue Cross Blue Shield HMO Insurance Company and Northlake Regional Medical Center.


Alexander has found his passion actively practicing aesthetics, tissue rejuvenation, sexual restoration and anti-aging. In some cases the conductive and the sensorineural elements may have started simultaneously ( severe head injury affecting both the inner ear and the middle ear ). Despite meticulous surgical care, the inner ear also can be traumatized readily and made more susceptible to infection.
The best way to approximate the conductive and the sensorineural components of a hearing loss is to perform all possible tests for estimating the patients sensorineural potential or cochlear reserve.
If the sensorineural component is slight, the surgical prognosis is good, and under favorable circumstances the hearing may approximate the level of the bone conduction. In certain cases, central hearing loss may mimic peripheral causes of deafness, including occupational deafness.
However, if one gets the persons attention by calling his or her name before speaking, the first sentence will not be missed, and hearing is normal. Tinnitus is a characteristic feature of hysterical deafness, and patients often claim that the noise is unbearable.
Too spectacular an improvement with a hearing aid, especially when the patient has set the controls at minimal amplification, or a sudden disproportionate improvement in hearing after a simple procedure such as drum massage or insufflating the eustachian tube.
Such a patient even may deny hearing shouts directed at the bad ear in spite of the good hearing in the opposite ear. His organizational and leadership skills are refined having been the lead physician at the Goodwill Games, World University Games, and one of an 8 member team that developed and coordinated the events of the International Drug Testing Program for the 1996 Olympic Games. In surgical trauma to the inner ear, high-frequency hearing loss often falls below the preoperative level, and the patient may complain of reduced discrimination though his pure-tone threshold is improved. However, the discrimination is not improved much, even after correction of the conductive defect. The chief function of the auditory cortex is to interpret and to integrate these impulses and to provide the listener with the exact meaningful information intended by the speaker, or to permit the listener to react appropriately to the actual implication of the sound. The spheroid cells of the superior ventral cochlear nucleus (SVCN) show an anatomical frequency gradient, low ventral to high dorsal.
Treatment : altering the listening environment to obtain the best possible signal-to-noise ratio.
Hearing acuity usually varies, depending on the patients emotional state at the time of testing. In complete deafness the presence of cochlear nerve reflexes with loud noises indicates either malingering or hysteria. If the patient hears and discriminates well when speech is made louder, then the conductive element probably is a major cause of the hearing difficulty, and there is a good chance that surgery will improve the hearing.
A similar adverse effect is noted in the ear opposite the tumor when certain words are interrupted periodically or accelerated. He has treated patients in a variety of settings including hospitals, skilled nursing facilities, outpatient rehabilitation centers, and in patient’s homes.
He has worked with individuals of all ages with a broad spectrum of injuries and illnesses. Labyrinthitis Otosclerosis was at one time thought to retain its purely conductive character for years; today it is recognized that this condition develops sensorineural impairment in same cases.



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