Postlingual hearing loss definition un,names of earring closures today,ear nose throat doctor portland oregon 5k,homeopathic treatment for tinnitus zumbido - PDF 2016

Author: admin, 23.05.2014. Category: Severe Tinnitus Symptoms

Top of pageAbstractHearing loss is an etiologically diverse condition with many disease-related complications and major clinical, social, and quality of life implications. Because conversational speech is at approximately 50 to 60 dB hearing level (HL), calculating functional impairment on the basis of pure tone averages can be misleading. As the rate of acquired hearing loss secondary to environmental causes decreases and improvements in the diagnosis of abnormalities occur, the significance of genetic factors that lead to deafness increases. However, milder but still clinically significant degrees of unilateral or bilateral hearing loss occur in an additional one to two infants.5,6 More than 50% of all prelingual deafness cases are hereditary in nature, with the remaining 40% to 50% of cases secondary to environmental factors such as infectious or iatrogenic causes (Fig.
Smith, MD, Molecular Otolaryngology Research Laboratories, Department of Otolaryngology-Head and Neck Surgery, 5270 CBRB, The University of Iowa, Iowa City, IA 52242. Advancements in molecular biology have led to improved detection and earlier intervention in patients with hearing loss.
2).7,8 The majority of genetic hearing loss diagnosed in infancy and early childhood is autosomal recessive in inheritance and nonsyndromic. Subsequently, earlier implementation of educational services and cochlear implant technology in patients with profound hearing loss now results in superior communication skills and enhanced language development.
Radiology with computed tomography is helpful and demonstrates a dilation of the internal auditory meatus with an abnormal communication between the subarachnoid space and the cochlear endolymph. The causative gene for DFN3 is POU3F4.69Additional X-linked nonsyndromic hearing loss phenotypes include profound prelingual hearing loss characteristic of both DFN2 and DFN4.
Indeed, interactions between environmental triggers and an individual's genotype can contribute to the development of hearing loss.
In two families, a homoplasmic mutation at nt1555 (A-to-G) in the mitochondrial MTRNR1 gene has been reported. Accessed 24 January 2007.Full figure and legend (202K)In the investigation of hearing loss, genetic forms must be distinguished from acquired (nongenetic) causes.
This mutation is also present in persons with aminoglycoside-induced ototoxic hearing loss.
Perhaps most noteworthy is hearing loss from asymptomatic congenital cytomegalovirus infection.
Two other families with maternally inherited nonsyndromic hearing loss have been identified with heteroplasmy for an A-to-G transition at nt7445 of the MTTS1 gene.
Molecular genetic tests are available for many types of syndromic and nonsyndromic deafness, although often only on a research basis. Clinically, testing of GJB2 and GJB6 plays a prominent role in diagnosis and genetic counseling because mutations in these genes account for more than 50% of severe-to-profound autosomal recessive nonsyndromic deafness in many world populations. However, an individual's susceptibility to hearing loss most likely reflects an environmental-genetic interaction.Age-related hearing loss (presbycusis) is acquired and characterized by diminished hearing sensitivity and speech comprehension in noisy environments, slowed central processing of acoustic information, and impaired localization of sound.
The following clinical assessments are recommended.Family historyA three-generation family history with attention to other relatives with hearing loss and associated findings should be obtained.
Affected persons may have difficulty in conversation, music appreciation, orientation to alarms, and participation in social activities.15 The aging population in the Western world and the high prevalence of presbycusis combine to make age-related hearing loss a common social and health challenge. Documentation of relevant findings in relatives can be accomplished through direct examination of those individuals or through review of their medical records, including audiograms, otologic examinations, and DNA-based testing with appropriate consent.Physical examinationAll persons with hearing loss of unknown cause should be evaluated for features associated with syndromic deafness. Genetic counseling and risk assessment are dependent on the accurate determination of the specific genetic diagnosis. Important features include branchial cleft pits, cysts or fistulae, preauricular pits, telecanthus, heterochromia iridis, white forelock, pigmentary anomalies, high myopia, pigmentary retinopathy, goiter, and craniofacial and skeletal anomalies. Neurologic examination of vestibular function with an ophthalmologic examination should be completed. EHDI programs aim to screen neonates for hearing loss immediately after birth or before hospital discharge. The variable expressivity associated with syndromic deafness requires careful physical examination of the proband and other family members to make a correct diagnosis.Audiologic findingsEstablishing the diagnosis of a child or adult with presumed hereditary hearing loss can be difficult. One must both objectively assess auditory function and subjectively determine how a person processes auditory information, that is, hears. Their deafness is not considered to be a pathology or disease to be treated or cured.Hard of hearing is a term that is more functional than audiologic.
Physiologic tests, such as the ABR and the EOAE, are important objective determinants of the functional status of the auditory system and can be performed at any age. It is used by the Deaf to signify that a person has some usable hearing: anything from mild to severe hearing loss. In the first phase, newborns are screened with evoked otoacoustic emissions (EOAE) or auditory brainstem response (ABR) to detect permanent bilateral or unilateral sensory or conductive hearing loss averaging 30 to 40 dB HL or more in the frequency region important for speech recognition. Audiometry refers to subjective testing that consists of both behavioral testing and pure tone measurement of air and bone sound conduction.ABR uses sound stimuli in the form of clicks to evoke electrophysiologic responses that originate in the eighth cranial nerve and auditory brainstem. In the second phase, all infants who do not pass the initial screen are evaluated with a series of diagnostic audiologic tests, preferably before the age of 3 months. In contrast, sensorineural hearing loss results from the malfunction of inner ear structures such as the cochlea.
Mixed hearing loss is a combination of both conductive and sensorineural hearing loss, and central auditory dysfunction results from damage or dysfunction at the level of the eighth cranial nerve, auditory brainstem, or cerebral cortex.OnsetPrelingual hearing loss is present before speech develops. Skin electrodes measure if the auditory response is phase locking to alterations in a continuous tonal stimulus. All congenital hearing loss is prelingual, but not all prelingual hearing loss is congenital. The continuous signal denotes that the average sound pressure level delivered is higher than that achieved with ABR, which uses repetitive click stimuli.


Postlingual hearing loss occurs after the development of normal speech.SeverityHearing is measured in decibels (dB). This difference allows for auditory steady-state response testing to provide a measure of hearing sensitivity in children who demonstrate no response to ABR testing.EOAEs are sounds that originate within the cochlea and are measured in the external auditory canal using a probe with a microphone and transducer. The threshold or 0 dB mark for each frequency refers to the level at which normal young adults perceive a tone burst 50% of the time. It consists of variable degrees of prelingual nonprogressive sensorineural hearing loss accompanied by pigmentary abnormalities of the skin, hair (white forelock), and eyes (heterochromia iridis). EOAEs principally reflect the activity of the outer hair cells of the cochlea across a broad frequency range. Green GE, Scott DA, McDonald JM, Woodworth GG, et al.Carrier rates in the midwestern United States for GJB2 mutations causing inherited deafness. Hearing is considered normal if an individual's thresholds are within 15 dB of normal thresholds. Because affected persons may dye their hair, the presence of a white forelock should be specifically sought in the history and physical examination. EOAEs are only present in ears with hearing sensitivity better than 40 to 50 dB HL.Immittance testing includes tympanometry, testing of acoustic reflex thresholds, and acoustic reflex decay. Immittance audiometry evaluates the peripheral auditory system, including middle ear pressure, tympanic membrane mobility, eustachian tube function, and mobility of the middle ear ossicles.Behavioral testing includes behavioral observation audiometry and visual reinforcement audiometry.
WS I and WS II share many features but have an important phenotypic difference; WS I is characterized by the presence of a lateral displacement of the inner canthus of the eye, which is known as dystopia canthorum, whereas in WS II this feature is absent. Behavioral observation audiometry is used in infants from birth to age 6 months and is highly dependent on the skill of the tester.
In WS III upper-limb abnormalities are present, and in WS IV, Hirschsprung disease is present. Harris S, Ahlfors K, Ivarsson S, Lernmark B, et al.Congenital cytomegalovirus infection and sensorineural hearing loss. Visual reinforcement audiometry is used in children from age 6 months to 2.5 years and may provide a reliable, complete audiogram. Hicks T, Fowler K, Richardson M, Dahle A, et al.Congenital cytomegalovirus infection and neonatal auditory screening. The syndrome is common, and three forms are recognized based on the precise molecular genetic defect involved. Intensity or loudness is measured in decibels, defined as the ratio between two sound pressures.
The average threshold for a normal hearing adult is between 0 and 20 dB HL, whereas sound at 120 dB HL is so loud that it can cause pain. Speech reception thresholds and speech discrimination are both assessed during pure-tone audiometric testing.Air conduction audiometry presents sounds through earphones with thresholds dependent on the condition of the external ear canal, middle, and inner ear. Bone conduction audiometry presents sounds through a vibrator placed on the mastoid bone or forehead, bypassing the external and middle ears. Ogawa H, Baba Y, Suzutani T, Inoue N, et al.Congenital cytomegalovirus infection diagnosed by polymerase chain reaction with the use of preserved umbilical cord in sensorineural hearing loss children.
Impairment primarily begins in the third decade, concomitant with the growth of a vestibular schwannoma, and is generally unilateral and gradual but may be bilateral and sudden.38 A retrocochlear lesion can often be diagnosed by audiologic evaluation, although the definitive diagnosis requires magnetic resonance imaging with gadolinium contrast.
If a child is cooperative, a complete frequency-specific audiogram for each ear can be obtained. In conventional audiometry, the individual indicates when the sound is heard and is used to test individuals age 5 years and older.An audioprofile refers to the recording of several audiograms on a single graph (Fig. Delayed organification of iodine by the thyroid can be documented by a perchlorate discharge test. Year 2000 position statement: principles and guidelines for early hearing detection and intervention programs.
The hearing loss is associated with an abnormality of the bony labyrinth (Mondini dysplasia or dilated vestibular aqueduct) that can be diagnosed by computed tomography of the temporal bones (Fig. The audiograms may be from one individual at different times, but more frequently they are from different members of the same family that segregate for deafness in an autosomal dominant trend. Plotting several audiograms with respective ages on the same graph allows for an age-related progression of hearing loss to be appreciated within a particular family. Baldwin CT, Hoth CF, Amos JA, da-Silva EO, et al.An exonic mutation in HuP2 paired domain gene causes Waardenburg's syndrome. The composite picture is often characteristic of a specific genetic cause of autosomal dominant nonsyndromic hearing loss. Affected individuals are born with sensorineural hearing loss and early in life develop retinitis pigmentosa, a progressive degeneration of the retina that leads to loss of night vision, restriction of visual fields, and blindness by adolescence. Usher syndrome affects more than 50% of the deaf-blind in the United States.44 It is of significance to note that the visual impairment from retinitis pigmentosa is usually not apparent in the first decade, thus making funduscopic examination before 10 years of age of limited utility. However, electroretinography can identify abnormalities in photoreceptor function in children as young as 2 to 4 years of age. The presence or absence of hearing loss in other family members should be documented by formal audiometric testing. During the second decade, night blindness and loss of peripheral vision become evident and inexorably progress.Usher syndrome is both phenotypically and genotypically heterogeneous.
Individuals with progressive hearing loss should be evaluated for Alport syndrome, Pendred syndrome, and Stickler syndrome.
Three types are recognized on the basis of the degree of hearing impairment and vestibular function.


Testing should also include temporal bone computed tomography.Sudden or rapidly progressive hearing loss can present with temporal bone anomalies or be secondary to a neoplasm. Usher syndrome type I is characterized by congenital severe-to-profound sensorineural hearing loss and vestibular dysfunction.
Furthermore, immunologic diseases, trauma, infections such as syphilis and Lyme disease, and metabolic, neurologic, or circulatory disturbances may also lead to sudden hearing loss.Laboratory and radiologic assessmentA thorough history and physical examination will support specific laboratory evaluations that are directed toward the suspected diagnosis.
Affected persons find traditional sound amplification ineffective and usually communicate manually.
This includes an immunoglobulin-M antibody assay in the first few years of life to assess for intrauterine infections such as cytomegalovirus and hemoglobinopathy testing that may be associated with sensorineural hearing loss. Because of the vestibular deficit, developmental motor milestones for sitting and walking are generally achieved later than normal. Usher syndrome type II is characterized by congenital mild-to-severe sensorineural hearing loss and normal vestibular function. Hearing aids provide effective sound amplification for these persons, and their communication is usually oral. Although molecular genetic testing is available for a number of these genes, the large size of many of these genes and their relatively small contribution to deafness make it impractical to offer such testing on a clinical basis at this time.Prenatal testingPrenatal diagnosis for some forms of hereditary hearing loss is technically possible by analysis of DNA extracted from fetal cells. Fetal material can be obtained by amniocentesis at 15 to 18 weeks' gestation or chorionic villus sampling at 10 to 12 weeks' gestation. Gestational age is expressed as menstrual weeks calculated either from the first day of the last normal menstrual period or by ultrasound measurements. Although a screening electrocardiogram is not highly sensitive, it may be suitable for screening deaf children. It is important to note that for optimal evaluation, the deafness-causing allele(s) of a deaf family member should be identified before prenatal testing is performed.Requests for prenatal testing for conditions such as hearing loss are not common. Differences in perspective may exist among medical professionals and within families regarding the use of prenatal testing, particularly if the testing is being considered for the purpose of pregnancy termination rather than early diagnosis. Although most centers would consider decisions about prenatal testing to be the choice of the parents, careful discussion of these issues is appropriate. Biotin covalently binds to four carboxylases that are essential for gluconeogenesis, fatty acid synthesis, and catabolism of several branched-chain amino acids. If biotinidase deficiency is not recognized and corrected by daily addition of biotin to the diet, affected persons develop neurologic features such as seizures, hypertonia, developmental delay, ataxia, and visual problems. Many deaf people are interested in obtaining information about the cause of their own deafness, including information on medical, educational, and social services rather than information about prevention, reproduction, or family planning. Furthermore, in at least 75% of children who become symptomatic, sensorineural hearing loss develops and can be both profound and persistent even after treatment is initiated.48 Cutaneous features are also present and include a skin rash, alopecia, and conjunctivitis.
With treatment that consists of biotin replacement, the neurologic and cutaneous manifestations resolve; however, the hearing loss and optic atrophy are usually irreversible. Therefore, if a child presents with episodic or progressive ataxia and progressive sensorineural deafness, with or without neurologic or cutaneous symptoms, biotinidase deficiency should be considered.
It may also be necessary to incorporate the expertise of an educator of the Deaf, a neurologist, and a pediatric ophthalmologist.
Sequential audiologic examinations are essential to document the stability or progression of the hearing loss and to identify and treat superimposed hearing losses, such as middle ear effusion.If an individual or the parents of a child raise concern about the possibility of hearing loss or, if the child is young, and there is concern regarding delayed speech development or poor social interaction, the initial evaluation should include a detailed history to explore possible acquired forms of hearing loss.
A family history with pedigree construction, and a thorough otolaryngologic examination with particular attention to otoscopy and physical examination focusing on features that might suggest a syndromic type of deafness should also be performed.An accurate test to quantify hearing acuity is paramount, and if one is not obtained, a return appointment should be scheduled as soon as possible to complete this part of the evaluation. Hearing loss usually does not manifest before the first decade of life, and the progressive myopia secondary to anterior lenticonus is considered by some authors to be sufficient to diagnose Alport syndrome.52 Autosomal dominant, autosomal recessive, and X-linked inheritance are described. On the basis of the data recorded from the initial evaluation, more specific tests can be requested that may include mutation screening of genes implicated in deafness.In contrast with many other medical conditions, the management and treatment of hearing loss largely involves the social welfare system and incurs a growing cost to society. For a child with prelingual severe to profound sensorineural hearing loss, the total lifetime cost of hearing loss includes special education services, medical expenses, and the purchase of assisted devices, altogether exceeding U.S.
Thus, an important component of the evaluation is to determine which avenue of habilitation best suits the individual.
Interventional services should begin before the age of 6 months for all infants, and possible treatments include hearing aids, vibrotactile devices, or cochlear implantation, which are now considered in children aged more than 12 months with severe-to-profound hearing loss.Although decreased cognitive skills and performance in mathematics and reading are associated with deafness, examination of persons with hereditary hearing loss has shown that these deficiencies are not intrinsically linked to the cause of the deafness. Loci for genes inherited in an autosomal dominant manner are referred to as DFNA, loci for genes inherited in an autosomal recessive manner are referred to as DFNB, and loci for genes inherited in an X-linked manner are referred to as DFN. For example, assessment of cognitive skills in individuals with connexin 26-related deafness reveals a normal Hiskey intelligence quotient and normal reading performance after cochlear implantation.
The condition is extremely heterogeneous, with multiple genes implicated in its pathogenesis. Audioprofiles may be distinct and therefore useful in predicting candidate genes for mutation screening. Because this risk can be minimized by avoidance, persons with documented hearing loss should be counseled appropriately.Top of pagePREVENTION OF HEARING IMPAIRMENTAn ideal therapy has not yet arrived to correct hearing loss through replacement or regeneration of inner and outer hair cells.
For example, mutations in WFS1 are found in 75% of families segregating autosomal dominant nonsyndromic hearing impairment that initially affects the low frequencies while sparing the high frequencies (Fig. These audiograms may be from one individual at different times, but frequently they are from different members of the same family segregating deafness usually in an autosomal dominant fashion.



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