Audiology 101: An Introduction To Audiology For .

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ANATIONALRESOURCECENTERGUIDE FORFOREARLYHEARINGHEARINGASSESSMENTDETECTION& &MANAGEMENTINTERVENTIONChapter 5Audiology 101:An Introductionto Audiology forNonaudiologistsTerry Foust, AuD, FAAA, CC-SLP/A; & Jeff Hoffman, MS, CCC-AParents of youngchildren who areidentified as deaf or hardof hearing (DHH) aresuddenly thrust into aworld of new conceptsand a bewildering arrayof terms.IntroductionParents of young children who areidentified as deaf or hard of hearing(DHH) are suddenly thrust into aworld of new concepts and a bewilderingarray of terms. What’s a decibel or hertz?What does sensorineural mean? Is amoderate hearing loss one to be concernedabout, since it’s only moderate? What’sa tympanogram or a cochlear implant?These are just a few of the many questionsthat a parent whose child has beenidentified as DHH may have. In additionto parents, questions also arise fromprofessionals and paraprofessionals whowork in the field of early hearing detectionand intervention (EHDI) and are notaudiologists. The purpose of this chapteris to provide basic answers to these andother important questions about the fieldof audiology.What is an audiologist?An audiologist is a specialist in hearingand balance who typically works ineither a medical, private practice, or aneducational setting. The primary roles ofan audiologist include the identificationand assessment of hearing and balanceproblems, the habilitation or rehabilitationof hearing and balance problems, and theprevention of hearing loss. When workingwith infants and young children, theprimary focus of audiology is hearing.Audiologists are licensed by the state inwhich they practice and may be membersof the American Speech-LanguageHearing Association (ASHA), AmericanAcademy of Audiology (AAA), Academyof Doctors of Audiology (ADA), or theEducational Audiology Association(EAA). Some audiologists hold theChapterfor5 NonaudiologistsAudiology 101: 5-1eBook Chapter 5 An IntroductioneBookto Audiology

A RESOURCE GUIDE FOR EARLY HEARING DETECTION & INTERVENTIONAmerican Board of Audiology’s PediatricAudiology Specialty Certification. Othersmay hold the ASHA Certificate of ClinicalCompetence in Audiology (CCC-A).Several online search tools are available tofind an audiologist: Early Hearing Detection andIntervention Pediatric Links toServices (EHDI-PALS)Early Hearing Detection andIntervention (EHDI) program, alsoknown as the Newborn HearingScreening Program, in each stateASHAAAAWhat are the parts of the ear?A basic understandingof the parts of theauditory system andhow they work is helpfulto understandingthe different types ofhearing loss.A basic understanding of the parts ofthe auditory system and how they workis helpful to understanding the differenttypes of hearing loss. There are four mainparts of the auditory system: Outer earMiddle earInner earCentral auditory systemEach part plays an important role intransferring and processing sound, so thatthe brain can recognize and interpret whata particular sound means.The outer ear consists of three major parts: PinnaEar canalEardrum (tympanic membrane)and fiber tissue. It is attached to the wall ofthe ear canal and also to one of the bonesof the middle ear. The eardrum seals themiddle ear from the environment. Soundvibrates the eardrum and is changed tomechanical energy.The middle ear is a hollow space thatis separated from the ear canal by theeardrum and contains the three smallestbones in the body. Sounds travel throughthe outer ear and are transferred to theinner ear by these bones (ossicles). Thethree bones are the hammer (malleus),anvil (incus), and stirrup (stapes). Theyare connected by ligaments, and two ofthe bones have tiny muscles attached. Theshape and arrangement of the ossiclesincreases the strength of the mechanicalenergy. When loud sounds are present,the tiny muscles contract and reduce thestrength of those sounds. This helps protectthe ear from damage due to loud sounds.The Eustachian tube is also part ofthe middle ear system and connectsthe middle ear space to the back ofthe throat. The Eustachian tube isnormally closed but opens periodicallyto keep the air pressure in the middleear space the same as the surroundingenvironment. An example of theEustachian tube working occurs whena yawn or a swallow “unstuffs the ears”Figure 1Anatomy of the AuditorySystemThe pinna is the part of the ear that wesee and contributes slightly to locatinga sound. The ear canal, which is aboutan inch long and S-shaped, channelssound toward the eardrum. The ear canalproduces earwax which helps to cleanthe ear canal of debris. The last part ofthe outer ear is the eardrum which isthe boundary between the outer and themiddle ears. The eardrum is a very thinmembrane, consisting of layers of skineBook Chapter 5 Audiology 101: 5-2

NATIONAL CENTER FOR HEARING ASSESSMENT & MANAGEMENTIf a child is born with asignificant malformationof the outer and/ormiddle ear that preventsor reduces the soundsbeing conducted to theinner ear, surgery maybe possible. The child’shearing ability may ormay not improve aftersurgical treatment.when flying in an airplane. Sometimesthe tissues of the Eustachian tubebecome swollen from a cold orupper respiratory infection, and itdoesn’t open and close well—causingproblems in the middle ear. It’spossible for infection to spread fromthe nose and throat area through theEustachian tube to the middle ear,which is one of the causes of middleear infections.What are the types ofhearing loss?The third part of the auditory system is theinner ear. The inner ear has two sections:one that is responsible for balance andthe other for hearing. The hearing part ofthe inner ear is the cochlea. The cochleais a snail-shaped space in the skull thatcontains very tiny structures that convertmechanical energy into electrical impulsesneeded for the nervous or central auditorysystem. The cochlea is divided by tissuestructures into three channels, each ofwhich is filled with fluid. There are manythousands of tiny hair cells (stereocilia)that are embedded in the tissue thatdivides the three sections. The hair cellsbend slightly in response to different kindsof sounds, depending on where they arelocated in the cochlea. When bent, thehair cells create electrical signals that arethen sent to the central auditory nervoussystem.One of the ways that an audiologistdescribes a hearing loss is by how manyears are involved. If a hearing loss is onlyin one ear, the loss is called a unilateral(one-sided) hearing loss. If there is hearingloss in both ears, it is described as abilateral (two-sided) hearing loss.The last part of the auditory systemconsists of the auditory nerve and thecentral auditory system in the brain. Theelectrical nerve impulses produced in thecochlea by the hair cells are transmittedand processed along the auditory nervethat consists of about 25,000 nerve fibers.The signal continues through the brainstem to the auditory cortex of the brain. Itis in the cortex that sounds are interpretedbased on experience and association andthat meaning is assigned to sounds thattravel through the outer, middle, and innerears.Auditory Transduction by Brandon Pletschis an excellent 7-minute animation onYouTube about the different structures ofthe auditory system, how they work, andhow each contributes to hearing.Hearing losses can be categorized by whenthey occur. A hearing loss that is present atbirth is called a congenital hearing loss. Buthearing loss can and does occur at any timeand can be called later-onset or acquired. Ifa hearing loss continues to get worse, it iscalled a progressive hearing loss.The different types of hearing loss areprimarily based on what part of the ear ispreventing a sound from being transferredand processed effectively.Conductive Hearing LossA conductive hearing loss occurs becauseproblems in the outer and/or middle earkeep the sound from being “conducted”well. Conductive hearing losses can beeither temporary (transient) or permanent.Medical treatment of the underlying causeof the temporary conductive hearing lossmay result in the hearing returning tonormal or near normal. For example, ifthe ear canal is plugged with earwax or anobject of some sort, some hearing loss willoccur until the blockage is removed. Theamount of hearing loss would be similar tohaving an earplug in your ear canal.At least 80% of children have three or moreepisodes of ear infections (otitis media)before 3 years of age (Roberts & Hunter,2002). These ear infections can be painful,and if the middle ear space fills with fluid,a temporary conductive hearing loss canoccur. Middle ear infections left untreatedcan cause some other middle ear problemsthat may result in a permanent loss.If a child is born with a significantmalformation of the outer and/or middleeBook Chapter 5 An Introduction to Audiology for Nonaudiologists 5-3

A RESOURCE GUIDE FOR EARLY HEARING DETECTION & INTERVENTIONear that prevents or reduces the soundsbeing conducted to the inner ear, surgerymay be possible. The child’s hearing abilitymay or may not improve after surgicaltreatment.Sensorineural (or Sensory) Hearing LossA sensorineural (or sensory) hearing lossfrom problems in the cochlea or innerear is almost always permanent. There aremany different causes of a sensorineuralhearing loss. Some losses can be geneticor syndromic—the result of somemedications, infections, high fevers, orhead trauma.Nationally, almost onethird of the newbornswho don’t pass thenewborn hearingscreening aren’t gettingthe recommendedfollow-up evaluations.Auditory neuropathy spectrumdisorder (ANSD) describes a disorderedtransmission of the electrical signalalong the acoustic nerve. ANSD—sometimes called auditory neuropathy/dyssynchrony—is relatively rare in thewell-baby population and somewhatmore prevalent in babies who havespent time in the neonatal intensive careunit.Mixed Hearing LossA mixed hearing loss has botha conductive component and asensorineural component. Theconductive component is the result ofa problem in the outer and/or middleear, while the sensory or sensorineuralportion results from a problem in theinner ear.Central Hearing LossCentral auditory processing refers to howwell the central nervous system transmitsand uses auditory information. Disordersof central auditory processing can includeproblems with determining where a soundis coming from and excessive difficultyunderstanding speech and auditorysignals in poor listening conditions,such as noisy settings. These disordersmay coexist with other disorders, suchas language impairment and learningdisorders, but is not the result of thosedisorders.How often does hearing lossoccur in young children?Many children have a temporaryconductive hearing loss due to earinfections, but how many have apermanent hearing loss? Each year, stateEHDI programs send the results of theirnewborn hearing screening programto the Centers for Disease Control andPrevention (CDC). In 2013, over 97% ofthe babies born in the United States hadtheir hearing screened within the first fewdays of life, and 5,253 of them were bornwith a permanent congenital hearing loss.This is a prevalence of 1.5 per thousand(15 of every 10,000) babies who receiveda newborn hearing screening. Those areonly the babies with a confirmed hearingloss. Nationally, almost one-third of thenewborns who don’t pass the newbornhearing screening aren’t getting therecommended follow-up evaluations, sothere are likely more congenital hearinglosses than are reported (CDC, 2015).About 40% of the newborns reported by thestate EHDI programs to the CDC for 2013had a unilateral hearing loss, while 60%of them had a bilateral hearing loss. Only14% of the babies born with a permanenthearing loss had a conductive hearing loss.About 62% of the permanent congenitalhearing losses reported to the CDC weresensorineural—by far the most commontype. Permanent congenital mixed hearinglosses are somewhat rare—about 8% of theresults reported to CDC in 2013. ANSDis also rare, accounting for only 5% of thepermanent congenital losses (CDC, 2015).Hearing loss can and does occur at any timein a person’s life. Eiserman et al. (2008)found that 1.5 per thousand (15 of every10,000) children up to 3 years of age who hadbeen screened using otoacoustic emissionstechnology in Early Head Start programshad a permanent hearing loss that hadn’tbeen identified earlier. Similarly, Bhatiaet.al. (2013) identified 2.5 per thousand (25of every 10,000) with a newly identifiedpermanent hearing loss in children birth to3 years in a screening program at federally-eBook Chapter 5 Audiology 101: 5-4

NATIONAL CENTER FOR HEARING ASSESSMENT & MANAGEMENTThe rate of permanenthearing loss continuesto climb as children getolder. It is estimatedthat as many as 14.9% ofschool-age children havea hearing loss.funded health clinics. Foust et al. (2013)found a similar rate of previously unidentifiedpermanent hearing loss 1.2 per thousand (12of every 10,000) children up to 5 years of agein federally-funded health clinics.In 2006, Morton and Nance publishedNewborn Hearing Screening - A SilentRevolution, which identified the causes ofpermanent hearing loss at birth and alsoat 4 years of age. As can be seen in Figure2, the incidence of permanent hearing lossat birth was nearly 2 per thousand (186per 10,000). By 4 years of age, as shownin Figure 3, the incidence of permanenthearing loss increased to about 3 perthousand (270 per 10,000).The rate of permanent hearing losscontinues to climb as children get older.It is estimated that as many as 14.9% ofFigure 2Causes of Hearing Lossat Birthschool-age children have a hearing loss(Niskar et al., 1998).What causes permanenthearing loss?Morton and Nance (2006) reported that amutation of the GJB2 gene was responsiblefor 21% of congenital permanent hearinglosses (see Figure 2). That, however,was only one of the genetic causes ofcongenital hearing loss. Causes of hearingloss associated with various syndromes,including Pendred’s syndrome, accountedfor 17% of congenital hearing loss, and anadditional 30% of hearing loss at birth wasdue to unspecified nonsyndromic geneticfactors. Overall, genetic factors accountedfor 68% of the congenital permanenthearing losses.Figure 3Causes of Hearing Lossat 4 Years of Age(Morton and Nance, 2006)eBook Chapter 5 An Introduction to Audiology for Nonaudiologists 5-5

A RESOURCE GUIDE FOR EARLY HEARING DETECTION & INTERVENTIONCongenital cytomegalovirus (CMV) is aleading cause of congenital hearing loss,accounting for 21% of the hearing lossespresent at birth. About half of childrenwith hearing loss due to CMV show othercomplications of congenital CMV, such asvision loss; small head size; or problemswith the liver, spleen, or lungs.By 4 years of age, over 50% of the hearinglosses were due to genetic factors (seeFigure 3). Congenital CMV still accountedfor about one-fourth of all permanenthearing losses, but there were some lateonset hearing losses due to CMV.Why is it important to screenhearing as early as possible?The sooner we can finda hearing problem, thesooner we can start tohelp, and the greaterthe success of languageand communicationdevelopment.It is critical to understand the importanceof discovering a hearing loss as soon aspossible. The sooner we identify a hearingproblem, the earlier the intervention tominimize the impact of the hearing loss andstrategies to maximize use of the remaininghearing sensitivity can be implemented.Simply stated, the sooner we can find ahearing problem, the sooner we can start tohelp, and the greater the success of languageand communication development.There are formal recommendations for theminimum ages and time periods for eachstep in the process for the identificationand diagnoses of hearing loss and thenecessary intervention and followup.The Joint Committee on Infant Hearing(JCIH, 2007) 2007 Position Statementrecommends the following newbornhearing screening guidelines:1 month. By 1 month of age, ahearing screening is completed.3 months. By 3 months of age,the child failing or referring ahearing screening will have acomplete diagnostic hearingevaluation with audiology andotolaryngology examinations.If a hearing loss is diagnosed, thechild will be fit with hearing aids as perthe parents choice.6 months. By 6 months of age, the childwill be enrolled in early interventionservices.Why are these targets so important?Because the rate of growth anddevelopment in the first year of ababy’s life is unmatched at any othertime during postnatal (after birth)development. These findings aresupported by various brain-imagingtechniques. Imaging studies, such asthis Positron Emission Tomographic(PET) scan (see Figure 4), show thatthe brain rapidly matures in an orderlyfashion during the first years of life.The orange-red color represents therapid growth from 1 month to 1 yearof age.Figure 4PET Scan Showing BrainMaturational Changes with AgeDuring this period, the infant brainis developing, and tiny synapses,which are biological electricalconnections, are forming. The amountof stimulation a child receives directlyimpacts the number of synapsesformed within the brain. This includesthe hearing, speech, and languagecenters of his or her brain. The creationof synapses is virtually complete afterthe first 3 years of life, thus thoseyears are the most important in braindevelopment.eBook Chapter 5 Audiology 101: 5-6

NATIONAL CENTER FOR HEARING ASSESSMENT & MANAGEMENTHow is a hearing lossdiagnosed?A hearing loss is detected by screening andtesting the auditory system through useof age-appropriate hearing tests. Hearingtests are used to determine four things:Significance of the Hearing LossThis means determining if the hearing lossis mild, moderate, moderate-to-severe,severe, or profound in nature.The Kind or Type of Hearing LossThis means determining if the hearing lossis caused by problems getting the soundinto the inner ear where it can then beheard (conductive hearing loss), or if itis a problem in the inner ear or beyondin the pathways of the auditory system(sensorineural hearing loss).Configuration of the Hearing LossThis means determining if hearing is better orworse at some pitches (frequencies). Hearingloss can be equal or flat across all the pitchesor better at either the low or high pitches.Make Intervention RecommendationsAnd decisions on treatment strategies thatmost benefit the child.What tests are used todetermine hearing?There are four primary tests for assessinghearing. They are:1234Immittance Audiometry. A test of middle ear function.These tests are sequential—moving fromthe outer part of the ear (peripheral) tomore internal (inner ear) to more centraland then to the whole auditory system. Itis important to remember that no one testcan stand alone. It takes an assessment of allthe elements of the auditory system—or a“battery of tests”—to confirm hearing status.Immittance Audiometry(Tympanometry and Acoustic Reflexes)Tympanometry is a measure of middleear function. It provides information onthe status (condition and function) of themiddle ear system. It evaluates the normaloccurring pressures of the middle earsystem (the pressure you feel change whenyou “pop” your ears), as well as the neededmobility or movement of the ear drum ortympanic membrane. It is conducted byplacing a small probe with a soft rubber tipin the ear canal and introducing pressurechanges along with a sound “tone” intothe ear canal. An abnormal result isconsistent with a problem in the transferof sound into the auditory system—knownas a conductive hearing loss. You mayhave experienced a conductive hearingloss when you had a head cold or an earinfection where sound was muffled.Tympanometry and measurement ofacoustic reflexes are a valuable componentof the audiological evaluation. In evaluatinghearing loss, immittance audiometrypermits a distinction between sensorineuraland conductive hearing loss. In addition, ina primary healthcare setting, tympanometrycan be helpful

problems in the middle ear. It’s possible for infection to spread from the nose and throat area through the Eustachian tube to the middle ear, which is one of the causes of middle ear infections. The third part of the auditory system is the inner ear. The inner ear has two sections: one that is responsible for balance and the other for hearing.

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