Guidance for the Clinician in Rendering Pediatric Care Clinical Report— Assessment in Infants and Children: Recommendations Beyond Neonatal Screening

Allen D. “Buz” Harlor, Jr, MD, Charles Bower, MD, THE abstract COMMITTEE ON PRACTICE AND AMBULATORY MEDICINE, THE SECTION ON OTOLARYNGOLOGY–HEAD AND NECK Congenital or acquired in infants and children has been SURGERY linked with lifelong deficits in speech and language acquisition, poor KEY WORD academic performance, personal-social maladjustments, and emo- hearing screening, hearing loss, tional difficulties. Identification of hearing loss through neonatal hear- ABBREVIATIONS ing screening, regular surveillance of developmental milestones, audi- AAP—American Academy of Pediatrics tory skills, parental concerns, and middle-ear status and objective OAE—otoacoustic emission ABR—auditory brainstem response hearing screening of all infants and children at critical developmental VRA—visual reinforced stages can prevent or reduce many of these adverse consequences. The guidance in this report does not indicate an exclusive This report promotes a proactive, consistent, and explicit process for course of treatment or serve as a standard of medical care. the early identification of children with hearing loss in the medical Variations, taking into account, individual circumstances may be appropriate. home. An algorithm of the recommended approach has been devel- This document is copyrighted and is property of the American oped to assist in the detection and documentation of, and intervention Academy of Pediatrics and its Board of Directors. All authors for, hearing loss. Pediatrics 2009;124:1252–1263 have filed conflict-of-interest statements with the American Academy of Pediatrics. Any conflicts have been resolved through a process approved by the Board of Directors. The American Academy of Pediatrics has neither solicited nor accepted any KEY POINTS commercial involvement in the development of the content of 1. Every child with 1 or more risk factors on the hearing risk assess- this publication. ment should have ongoing developmentally appropriate hearing screening and at least 1 diagnostic audiology assessment by 24 to 30 months of age. 2. Periodic objective hearing screening of all children should be per- formed according to the recommendations for preventive periodic health care.1 3. Any parental concern about hearing loss should be taken seriously and requires objective hearing screening of the patient. 4. All providers of pediatric health care should be proficient with pneu- www.pediatrics.org/cgi/doi/10.1542/peds.2009-1997 matic otoscopy and tympanometry. However, it is important to re- doi:10.1542/peds.2009-1997 member that these methods do not assess hearing. All clinical reports from the American Academy of Pediatrics automatically expire 5 years after publication unless reaffirmed, 5. Developmental abnormalities, level of functioning, and behavioral revised, or retired at or before that time. problems (ie, autism/developmental delay) may preclude accurate PEDIATRICS (ISSN Numbers: Print, 0031-4005; Online, 1098-4275). results on routine audiometric screening and testing. In this situa- Copyright © 2009 by the American Academy of Pediatrics tion, referral to an otorhinolaryngologist and a pediatric audiologist who has the necessary equipment and expertise to test infants and young children should be made. 6. The results of abnormal screening should be explained carefully to parents, and the child’s medical record should be flagged to facili- tate tracking and follow-up.

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7. Any abnormal objective screening TABLE 1 Recommendations for Preventive explicit process for the early identifi- result requires audiology referral Pediatric Health Care1 cation of children with hearing loss in and definitive testing. Stage Age Sensory the medical home. To assist in the de- Screening: 8. A failed infant hearing screening or 6. Hearing tection and documentation of and in- a failed screening in an older child Infancy Prenatal tervention for hearing loss, an algo- should always be confirmed by fur- Newborn a rithm of the recommended approach 3–5 d1 b with key points has been developed ther testing. By1mo b (Fig 1), as have several tables. 9. Abnormal results re- 2mo b 4mo b quire intervention and clinically 6mo b RISK INDICATORS FOR HEARING appropriate referral, including oto- 9mo b LOSS laryngology, audiology, speech- Early childhood 12 mo b b Some degree of hearing loss (Table 2) language pathology, genetics, and 15 mo 18 mo b is present in 1 to 6 per 1000 newborn early intervention. b 24 mo infants.5 Most children with congenital 30 mo b b hearing loss are potentially identifi- INTRODUCTION 3y 4y a able by newborn and infant hearing Failure to detect congenital or ac- Middle childhood 5 y a screening. However, some congenital quired hearing loss in children may re- 6y a 7y b hearing loss may not become evident sult in lifelong deficits in speech and 8y a until later in childhood. Hearing loss language acquisition, poor academic 9y b also can be acquired during infancy or a performance, personal-social malad- 10 y childhood for various reasons. Infec- Adolescence 11 y b justments, and emotional difficulties. 12 y b tious diseases, especially meningitis, Early identification of hearing loss and 13 y b are a leading cause of acquired hear- appropriate intervention within the 14 y b b ing loss. Trauma to the nervous sys- first 6 months of life have been demon- 15 y 16 y b tem, damaging noise levels, and oto- strated to ameliorate many of these 17 y b toxic drugs can all place a child at risk adverse consequences and facilitate 18 y b of developing acquired hearing loss.6,7 b language acquisition.2 Supportive evi- 19 y 20 y b Otitis media is a common cause of usu- dence is outlined in the Joint Commit- 21 y b ally reversible hearing loss. Certain tee on Infant Hearing’s “Year 2007 a To be performed. physical findings, historical events, Position Statement: Principles and b Risk assessment, with appropriate action to follow if and developmental conditions may in- positive. Guidelines for Early Hearing Detection dicate a potential hearing problem. and Intervention Programs,” which These conditions include, but are not was endorsed by the American Acad- All providers of pediatric health care limited to, anomalies of the ear and emy of Pediatrics (AAP).3 This evidence need to recognize children who are at other craniofacial structures, signifi- also is part of the rationale for the AAP risk of or who suffer from congenital or cant perinatal events, and global devel- statement “Newborn and Infant Hear- acquired hearing loss, be prepared to opmental or speech-language delays. ing Loss: Detection and Intervention,”4 screen their hearing, and assist the fam- All older infants and children should be which endorses universal hearing ily and arrange for proper referral and screened for risk factors involving hear- screening and reviews the primary ob- treatment by identifying available hear- ing problems. A summary of high-risk in- jectives, important components, and ing resources within their communities. dicators for hearing loss and develop- recommended screening methods and In addition, the pediatric health care pro- mental milestones are included in parameters that characterize an ef- fessional can play an important role in Tables 3 and 4, respectively. All infants fective universal hearing screening communication with the child’s school- with a risk indicator for hearing loss, re- program. Furthermore, the AAP teacher and/or nurse and special educa- gardless of surveillance findings, should statement “Recommendations for tion professionals to facilitate proper ac- be referred for an audiologic assess- Preventive Pediatric Health Care”1 commodation and education once a ment at least once by 24 to 30 months of promotes objective newborn hearing hearing deficit has been confirmed. age, even if the child passed the newborn screening as well as periodic hear- This clinical report replaces the previ- hearing screening. Children with risk in- ing screening for every child through ous 2003 clinical report and seeks to dicators that are highly associated with adolescence (Table 1). promote a proactive, consistent, and delayed-onset hearing loss, such as hav-

PEDIATRICS Volume 124, Number 4, October 2009 1253 Downloaded from www.aappublications.org/news by guest on September 29, 2021 FIGURE 1 Hearing-assessment algorithm within an office visit. CMV indicates cytomegalovirus; ENT, ear, nose, and throat. ing received extracorporeal membrane shown that only 50% of children with tal concern is of greater predictive oxygenation or having cytomegalovirus hearing loss are identified by the com- value than the informal behavioral ex- infection, should have more frequent au- prehensive use of such question- amination performed in the physi- diological assessments. Key point 1: Ev- naires.8,9 Key point 2: Periodic objective cian’s office.10 Parents often report ery child with 1 or more risk factors on hearing screening of all children suspicion of hearing loss, inattention, the hearing risk assessment should should be performed according to the or erratic response to sound before have ongoing developmentally appropri- recommendations for preventive peri- hearing loss is confirmed.11 One study ate hearing screening and at least 1 di- odic health care1 (Table 1). showed that parents were as much as agnostic audiology assessment by 24 to If a parent or caregiver is concerned 12 months ahead of physicians in iden- 30 months of age (Table 1). that a child might have hearing loss, tifying their child’s hearing loss.3 Key Although questionnaires and check- the pediatrician needs to assume that point 3: Any parental concern about lists are useful for identifying a child at such is true until the child’s hearing hearing loss should be taken seriously risk of hearing loss, studies have has been evaluated objectively. Paren- and requires objective hearing screen- ing of the patient.

TABLE 2 Definitions of Hearing Loss PHYSICAL EXAMINATION Hearing Definition Thorough physical examination is an Loss essential part of evaluating a child for Mild On average, the most quiet sounds that people can hear with their better ear are between 20 and 40 dB. People who suffer from mild hearing loss have some hearing loss. Findings on head and difficulties keeping up with conversations, especially in noisy surroundings. neck examination associated with po- Moderate On average, the most quiet sounds heard by people with their better ear are tential hearing loss include hetero- between 40 and 70 dB. People who suffer from moderate hearing loss have difficulty keeping up with conversations when not using a hearing aid. chromia of the irises, malformation of Severe On average, the most quiet sounds heard by people with their better ear are the auricle or ear canal, dimpling or between 70 and 95 dB. People who suffer from severe hearing loss will benefit skin tags around the auricle, cleft lip from powerful hearing aids, but often they rely heavily on lip reading, even or palate, asymmetry or hypoplasia of when they are using hearing aids. Some also use sign language. Adapted from: European Group on Genetics of Hearing Impairment. Martini A, ed. European Commission Directorate, the facial structures, and microcepha- Biomedical and Health Research Programme (HEAR) Infoletter 2, November 1996;8. ly.12 Hypertelorism and abnormal pig-

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TABLE 3 American Academy of Pediatrics Joint Committee on Infant Hearing Year 2007 Position Screenings should be conducted in a Statement3: Risk Indicators Associated With Permanent Congenital, Delayed-Onset, and/or Progressive Hearing Loss in Childhood quiet area where visual and auditory 1 Caregiver concerna regarding hearing, speech, language, or developmental delay. distractions are minimal. For children 2 Family historya of permanent childhood hearing loss. for whom screening is not possible be- 3 Neonatal intensive care of more than 5 days or any of the following regardless of length of cause of developmental level, referral a stay: ECMO , assisted ventilation, exposure to ototoxic medications (gentamicin and to a pediatric audiologist should be tobramycin) or loop diuretics (furosemide/Lasix), and hyperbilirubinemia19 that requires exchange transfusion. initiated for appropriate physiologic 4 In utero infections such as CMVa, herpes, rubella, syphilis, and toxoplasmosis. and/or behavioral audiological as- 5 Craniofacial anomalies, including those that involve the pinna, ear canal, ear tags, ear pits, sessment. Various tests performed by and temporal bone anomalies. 6 Physical findings, such as white forelock, that are associated with a syndrome known to audiologists are outlined in Table 5. include a sensorineural or permanent . 7 Syndromes associated with hearing loss or progressive or late-onset hearing lossa, such Tympanograms as neurofibromatosis, osteopetrosis, and ; other frequently identified syndromes include Waardenburg, Alport, Pendred, and Jervell and Lange-Nielson. Conductive hearing loss may be the 8 Neurodegenerative disordersa, such as Hunter syndrome, or sensory motor neuropathies, most common cause of infant hearing such as Friedreich ataxia and Charcot-Marie-Tooth syndrome. 15 9 Culture-positive postnatal infections associated with sensorineural hearing lossa, including screening failures. Objective middle- confirmed bacterial and viral (especially herpes viruses and varicella) meningitis. ear assessment can best be per- 10 Head trauma, especially basal skull/temporal bone fracturea that requires hospitalization. formed by tympanometry. Tympanom- 11 Chemotherapya. 12 Recurrent or persistent otitis media for at least 3 months. etry measures relative changes in Risk indicators that are marked with a are of greater concern for delayed onset hearing loss. ECMO indicates extracorporeal tympanic membrane movement as air membrane oxygenation; CMV, cytomegalovirus. pressure is varied in the external audi- Reproduced with permission from: American Academy of Pediatrics, Joint Committee on Infant Hearing. Pediatrics. 2007; 120(4):898–921. tory canal. Tympanograms (Fig 2) can most simply be classified as types A, B, and C depending on the curve shape rel- ative to 0 as the pressure is changed mentation of the skin, hair, or eyes ficient with pneumatic otoscopy and (www.audiologyonline.com/askexpert/ also may be associated with hearing tympanometry. However, it is impor- display࿝question.asp?question࿝idϭ451). loss, as in Waardenburg syndrome. tant to remember that these meth- The presence of renal abnormalities ods do not assess hearing. The presence of a type A, high-peaked (Alport syndrome), cardiac anomalies tympanogram significantly decreases (prolonged QT interval in Jervell and TOOLS FOR OBJECTIVE HEARING the probability that middle-ear effu- Lange-Nielsen syndrome), and other SCREENING sion is the cause of hearing loss. A type syndromes should also prompt evalu- In addition to universal newborn hear- B, flat tympanogram has the highest ation of hearing. Abnormalities of the ing screening and regular surveillance probability of the presence of middle- eardrum should alert the physician to of developmental milestones, auditory ear effusion or tympanic membrane the possibility of hearing loss. Ceru- skills, parental concerns, and middle- perforation, which are both likely to men impactions can obscure the tym- ear status, objective screenings for cause some degree of hearing loss. A panic membrane, preventing an accu- hearing loss should be performed pe- type C tympanogram, with a peak rate examination, and may cause riodically on all infants and children in shifted toward negative pressure, has hearing loss. Dense cerumen impac- accordance with the schedule outlined a low probability of middle-ear fluid tions should be removed before diag- in the AAP statement “Recommenda- and associated hearing loss. Type B nostic testing. A leading cause of ac- tions for Preventive Pediatric Health and C tympanograms require clinical quired hearing loss is otitis media with Care”1 (Table 1). The technology used correlation and possibly further evalu- effusion. Temporary hearing loss for hearing screening should be age ation and treatment. Traditionally, has been demonstrated during epi- appropriate. The child also should be tympanograms have been obtained by sodes of acute otitis media. The child comfortable with the testing situation; using low-frequency probe tones. with repeated or chronic otitis media young children may need preparation. These tones have been historically in- with effusion is at high risk of ac- A variety of objective tools have been accurate for infants younger than 6 quired hearing loss and should un- developed for screening tests. The months. The use of a high-frequency dergo comprehensive hearing evalu- choice of tool to use in screening de- probe tone (1000 Hz) was recently ation.13,14 Key point 4: All providers of pends on the child’s age, degree of co- shown to be a better measure of pediatric health care should be pro- operation, and available resources. middle-ear status in infants and young

PEDIATRICS Volume 124, Number 4, October 2009 1255 Downloaded from www.aappublications.org/news by guest on September 29, 2021 TABLE 4 Developmental Milestones in the First 2 Years of Life Mild degrees of motion artifact do not Milestone Average Age of Developmental Implications interfere with test results; however, Attainment, mo screening results are frequently influ- Gross motor enced by the presence of middle-ear Head steady in sitting 2.0 Allows more visual interaction Pull to sit, no head lag 3.0 Muscle tone pathologic abnormalities. The OAE test Hands together in midline 3.0 Self-discovery is an effective screening measure for Asymmetric tonic neck reflex gone 4.0 Child can inspect hands in midline middle-ear abnormalities and for mod- Sits without support 6.0 Increasing exploration erate or more severe degrees of hear- Rolls back to stomach 6.5 Truncal flexion, risk of falls Walks alone 12.0 Exploration, control of proximity to ing loss, because normal OAE re- parents sponses are not obtained if hearing Runs 16.0 Supervision more difficult thresholds are approximately 30- to Fine motor Grasps rattle 3.5 Object use 40-dB hearing levels or higher. The au- Reaches for objects 4.0 Visuomotor coordination tomated OAE screener provides a pass- Palmar grasp gone 4.0 Voluntary release fail report; no test interpretation by an Transfers object hand to hand 5.5 Comparison of objects Thumb-finger grasp 8.0 Able to explore small objects audiologist is required. The OAE test Turns pages of book 12.0 Increasing autonomy during book time does not further quantify hearing loss Scribbles 13.0 Visuomotor coordination or hearing threshold level. The OAE test Builds tower of 2 cubes 15.0 Uses objects in combination also does not assess the integrity of the Builds tower of 6 cubes 22.0 Requires visual, gross, and fine motor coordination neural transmission of sound from the Communication and language eighth nerve to the brainstem and, Smiles in response to face, voice 1.5 Child more active social participant therefore, will miss auditory neuropa- Monosyllabic babble 6.0 Experimentation with sound, tactile Inhibits to “no” 7.0 Response to tone (nonverbal) thy and other neuronal abnormalities. Follows 1-step command with gesture 7.0 Nonverbal communication Infants with such abnormalities will Follows 1-step command without 10.0 Verbal receptive language have normal OAE test results but ab- gesture (eg, “Give it to me”) Speaks first real word 12.0 Beginning of labeling normal auditory brainstem response Speaks 4–6 words 15.0 Acquisition of object and personal names (ABR) test results. A “failed” OAE test Speaks 10–15 words 18.0 Acquisition of object and personal names only implies that a hearing loss of Speaks 2-word sentences (eg, “Mommy 19.0 Beginning grammaticization, corresponds more than 30 to 40 dB may exist or that shoe”) with vocabulary of Ն50 words Cognitive the middle-ear status is abnormal. Stares momentarily at spot where 2.0 Lack of object permanence (out of sight, object disappeared (eg, yarn ball out of mind) Automated ABR dropped) Stares at own hand 4.0 Self-discovery, cause and effect One objective physiologic means of Bangs 2 cubes 8.0 Active comparison of objects screening hearing is the automated Uncovers toy (after seeing it hidden) 8.0 Object permanence ABR. This instrument measures co- Egocentric pretend play (eg, pretends 12.0 Beginning symbolic thought to drink from cup) chlear response in the 1- to 4-kHz Uses stick to reach toy 17.0 Able to link actions to solve problems range with a broadband click stimulus Pretend play with doll (gives doll bottle) 17.0 Symbolic thought in each ear. Many ABR screening in- Modified from: Behrman RE, Jenson HB, Kliegman R, eds. Nelson Textbook of Pediatrics. 16th ed. Philadelphia, PA: Saunders; struments incorporate built-in artifact 2003. rejection for myogenic, electrical, and environmental noise interference, which ensures that data collection is children. These tympanograms are the ear canal. These signals are gener- halted if testing conditions are unfa- generally not classified as A, B, or C but ated in response to an auditory stimu- vorable. The automated screener pro- rather as peak or no peak. lus, either clicks or tone bursts. The vides a pass-fail report; no test inter- signals may be detected with a very pretation by an audiologist is required. Evoked Otoacoustic Emissions sensitive microphone/probe system A “fail” report on an automated ABR im- Evoked otoacoustic emissions (OAEs) placed in the external ear canal. The plies a hearing level of worse than 40 dB. are acoustic signals generated from OAE test allows for individual ear as- Automated ABR can test each ear individ- within the cochlea that travel in a re- sessment, can be performed quickly at ually and can be performed on children verse direction through the middle-ear any age, and does not depend on of any age. Motion artifacts interfere space and tympanic membrane out to whether the child is asleep or awake. with test results. For this reason, the test

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TABLE 5 Audiologic Tests for Infants and Children Developmental Auditory Test/ Type of Measurement Test Procedures Advantages Limitations Age of Child Average Time All ages Evoked OAEs/10-min test Physiologic test specifically Small probe containing a Ear-specific results; not Infant or child must be relatively measuring cochlear sensitive microphone dependent on inactive during the test; not a (outer hair cell) is placed in the ear whether patient is comprehensive test of response to canal for stimulus asleep or awake; hearing, because it does not presentation of a delivery and response quick test time; assess cortical processing of stimulus; stimuli may be detection screening test sound; OAEs are very sensitive clicks (transient evoked to middle-ear effusions and OAEs) or tone pairs cerumen or vernix in the ear (distortion product canal OAEs) Birth to 9 mo Automated ABR/15-min Electrophysiologic Placement of electrodes Ear-specific results; Infant or child must remain test measurement of activity on child’s head detects responses not quiet during the test in auditory nerve and neurologic response to dependent on patient (sedation is often required); brainstem pathways auditory stimuli cooperation; not a comprehensive test of presented through screening test hearing, because it does not earphones or ear assess cortical processing of inserts 1 ear at a time sound 9 mo to 2.5 y VRA/15- to 30-min test Behavioral tests measuring Technique conditions the Assesses auditory When performed with speakers, responses of the child to child to associate perception of child; only assesses hearing of the speech and frequency- speech or frequency- diagnostic test. better ear; not ear specific; if specific stimuli specific stimuli with a VRA is performed with insert, presented through reinforcer, such as a earphones can rule out a speakers or insert lighted toy or video unilateral hearing loss earphones clips; VRA requires a calibrated, sound- treated room 2.5 to 4 y Play audiometry/ Behavioral test of auditory Child is conditioned to Ear-specific results; Attention span of child may limit 15–30 min thresholds in response respond when assesses auditory the amount of information to speech and stimulus tone is perception of child; obtained frequency-specific heard, such as to put screening or stimuli presented a peg in a pegboard diagnostic test. through earphones and/ or drop a block in a or bone vibrator box 4 y to adolescence Conventional audiometry/ Behavioral test measuring Patient is instructed to Ear-specific results; Depends on the level of 15- to 30-min test auditory thresholds in raise his or her hand assesses auditory understanding and response to speech and when stimulus is perception of patient; cooperation of the child frequency-specific heard screening or stimuli presented diagnostic test through earphones and/ or bone vibrator All ages Diagnostic ABR Electrophysiologic Placement of electrodes Ear-specific results; Infant or child must remain measurement of activity on child’s head multiple frequencies quiet during the test in auditory nerve and detects auditory are tested, creating a (sedation is often required); brainstem pathways stimuli presented map of hearing loss not a true test of hearing, through insert similar to an because it does not assess earphones 1 ear at a audiogram; cortical processing of sound time responses not dependent on patient cooperation; diagnostic test All ages Tympanometry Relative change in middle- Small probe placed in Tests for possible Not a test of hearing; depends ear compliance as air the ear canal and middle-ear pathology on ear canal seal; high- pressure is varied in the pressure varied in the and pressure- frequency tone probe needed external auditory canal ear canal equalization tube for infants younger than 6 mo function Adapted with permission from: Bachmann KR, Arvedson JC. Pediatr Rev. 1998;19(5):155–165. is performed best in infants and young motion artifact, sedation may be neces- ABR and OAEs are tests of auditory path- children while they are sleeping. If the sary. The ABR is currently used in many way structural integrity but are not true test cannot be performed because of newborn screening programs. tests of hearing. Even if ABR or OAE test

PEDIATRICS Volume 124, Number 4, October 2009 1257 Downloaded from www.aappublications.org/news by guest on September 29, 2021 Hearing thresholds at specific frequen- cies can be determined, and the degree of hearing loss can be assigned. If there are distractions or the room is not sound treated, pure-tone audiometry in the of- fice should be considered solely a screening test.

Play Audiometry Children 2 to 4 years of age are screened or tested more appropri- ately by play audiometry. These chil- dren are conditioned to respond to an auditory stimulus through play activi- ties, such as dropping a block when a sound is heard through earphones. Air-conduction hearing threshold lev- els of greater than 20 dB at any of these frequencies indicate possible hearing loss, and referral to a pediat- ric audiologist should be made.

Conventional Screening Audiometry For children aged 4 years and older, conventional screening audiometry can be used. The child is asked to raise his or her hand when a sound is heard. The test should be performed in a quiet environment using earphones, because ambient noise can affect test perfor- mance significantly, especially at lower frequencies (ie, 500 and 1000 Hz). Each ear should be tested at 500, 1000, 2000, and 4000 Hz. Air-conduction hearing threshold levels of greater than 20 dB at any of these frequencies indicate possi- ble hearing loss, and referral to a pedi- atric audiologist should be made. If the child does not pass the screen- ing, earphones should be removed and instructions carefully repeated to the child to ensure proper understanding and attention to the test and then re- FIGURE 2 screened with the earphones reposi- Tympanograms. Type A: normal. Type B: abnormal, needs medical attention. Type C, borderline normal; monitor; may need medical attention. tioned. A child whose repeat test shows hearing thresholds of greater results are normal, hearing cannot be havioral audiogram to be obtained. Be- than 20 dB at any of these frequencies, definitively considered normal until a havioral pure-tone audiometry remains especially if there is no pathologic ab- child is mature enough for a reliable be- the standard for hearing evaluation. normality of the middle ear on physical

1258 FROM THE AMERICAN ACADEMY OF PEDIATRICS Downloaded from www.aappublications.org/news by guest on September 29, 2021 FROM THE AMERICAN ACADEMY OF PEDIATRICS examination, should be referred for TABLE 6 Ten Ways to Recognize Hearing Loss: Adolescents (11- to 21-Year Visits) formal hearing testing. Key point 5: De- 1. Do you have a problem hearing over the telephone? velopmental abnormalities, level of 2. Do you have trouble following the conversation when two or more people are talking at the same time? functioning, and behavioral problems 3. Do people complain that you turn the TV volume up too high? (ie, autism/developmental delay) may 4. Do you have to strain to understand conversation? preclude accurate results on routine 5. Do you have trouble hearing in a noisy background? 6. Do you find yourself asking people to repeat themselves? audiometric screening and testing. In 7. Do many people you talk to seem to mumble (or not speak clearly)? this situation, referral to an otorhino- 8. Do you misunderstand what others are saying and respond inappropriately? laryngologist and a pediatric audiolo- 9. Do you have trouble understanding the speech of women and children? gist who has the necessary equipment 10. Do people get annoyed because you misunderstand what they say? Adapted from: National Institute on Deafness and Other Communication Disorders. Ten Ways to Recognize Hearing Loss. and expertise to test infants and young Bethesda, MD: National Institute of Health; 2006. NIH publication 01-4913. Available at: www.nidcd.nih.gov/health/hearing/ children should be made (Table 5). Key 10ways.asp. point 6: The results of abnormal screening should be explained care- frequency-specific hearing thresholds ing will only result in benefit for the fully to parents, and the child’s medi- by air and bone conduction in each ear patient if abnormal test results are cal record should be flagged to facili- separately. ABR is the gold standard confirmed and appropriate interven- tate tracking and follow-up. for determination of hearing thresh- tion is provided. Most studies that have It is important to remember that a olds in infants younger than 6 months evaluated the success rate of infant “fail” report on any 1 of a combination and in children who cannot be tested hearing screening programs have de- of tests warrants additional testing. It behaviorally. scribed a fairly high rate of failure to is also important to remember that Children as young as 6 to 24 months confirm a failed screen with definitive failure of speech, language, and hear- can be tested by means of visual rein- testing. A similar problem could also ing screening assessments warrants forced audiometry (VRA). This tech- occur in screening older infants and additional testing (Tables 6–9). nique conditions the child to associate children. Improving the physician’s in- speech or frequency-specific sound volvement not only in screening but Comprehensive Audiological with a reinforcement stimulus such also in arranging and confirming ap- Evaluation Using Physiologic and/ as a lighted toy or animated toy or propriate follow-up testing and inter- or Behavioral Testing video clips. VRA is performed by an vention is necessary to achieve opti- The ABR test may be used as a diagnos- audiologist with experience testing mal speech, language, and hearing. tic tool by audiologists for more defin- young children. This testing is not Key point 8: A failed infant hearing itive diagnosis of hearing loss. Usually readily applied in screening pro- screening or a failed screening in an performed in children in natural sleep grams, because infants younger older child should always be con- up to approximately 3 to 6 months of than 6 months’ developmental age firmed by further testing. Audiologists age and then under sedation for older cannot perform the task, and sound- may repeat the audiometric test as de- infants, diagnostic ABR can provide not treated rooms are needed. The re- scribed above in a sound booth and only a general level of hearing but also sults of VRA can approximate those using a variety of other tests. ABR can frequency-specific hearing data. Diag- of conventional audiometry. also be used for definitive testing of nostic ABR is performed with different Children with unilateral or mild hear- the auditory system. A diagnostic ABR frequency tone bursts and across ing loss also should be evaluated fur- is usually performed under sedation varying sound levels to effectively esti- ther. Studies have shown such chil- or general anesthesia in children aged mate an audiogram. Diagnostic ABR dren to be similarly at risk of adverse approximately 3 to 6 months and older. can also be performed with bone con- communication skills as well as diffi- The test is performed with frequency- duction to separate conductive from culties with social, emotional, and ed- specific stimuli and presentation lev- sensorineural hearing loss. Diagnostic ucational development.16 els to approximate hearing threshold ABR is often the definitive test used by levels. Diagnostic ABR provides infor- audiologists in children and infants FOLLOW-UP AND DIAGNOSTIC mation that is accurate enough to al- who are unable to cooperate with TESTING low for therapeutic intervention. Hear- other methods of hearing testing. Au- Key point 7: Any abnormal objective ing aids can be fitted with information diologic evaluation using ABR or audi- screening result requires audiology obtained from a diagnostic ABR. Audio- tory steady-state response provides referral and definitive testing. Screen- logic assessment and intervention is

PEDIATRICS Volume 124, Number 4, October 2009 1259 Downloaded from www.aappublications.org/news by guest on September 29, 2021 TABLE 7 Developmental/Behavioral Screening Tools Resource Description Age Range Where to Find General developmental screening tools Ages & Stages Questionnaire (ASQ) A series of 19 questionnaires used to screen 4–60 mo www.brookespublishing.com/tools/ infants and young children for asq/index.htm developmental delays during the first 5 y of life Ages & Stages Questionnaire: Social-Emotional A series of 19 questionnaires used to screen 6–60 mo www.brookespublishing.com/tools/ (ASQ:SE) infants and young children at risk for asqse/index.htm social or emotional difficulties, to identify behaviors of concern to caregivers, and to identify any need for further assessment Parents’ Evaluation of Developmental Status A method for detecting developmental and Birth to 8 y www.pedstest.com (PEDS) behavioral-emotional problems in children Parents’ Evaluation of Developmental Status- A collection of 6–8 items per age/encounter Birth to 11 y www.pedstest.com/dm Developmental Milestones (PEDS:DM) designed to replace informal milestones checklists with highly accurate items known to predict developmental status Autism-specific screening tools Checklist for Autism in Toddlers (CHAT) A screening tool for early detection of 18 to Ն24 mo www.autismresearchcentre.com/tests/ autism chat࿝test.asp Checklist for Autism in Toddlers (CHAT), Denver CHAT scoring modifications 18 to Ն24 mo Modifications Checklist for Autism in Toddlers-23 (CHAT-23) Combination of M-CHAT and CHAT items 16–86 mo Childhood Asperger Syndrome Test (CAST) A parental questionnaire to screen for 4–11 y www.autismresearchcentre.com/tests/ autism spectrum conditions cast࿝test.asp Modified Checklist for Autism in Toddlers 23-item scale pointing to express interest, 16–48 mo http://depts.washington.edu/dbpeds/ (M-CHAT) responsiveness to name, interest in Screening%20Tools/MCHAT.doc peers, showing behavior, response to joint attention, social imitation Pervasive Developmental Disorders Screening A parental questionnaire to screen for 18–48 mo www.pearson-uk.com/product.aspx? Test-II, Primary Care Screener (PDDST-II PCS) autism spectrum conditions nϭ1315&skeyϭ2960 Autism-specific or psychosocial screening tools Pediatric intake form from Bright Futures Questionnaire to help gather a general Birth to 21 y www.brightfutures.org/mentalhealth/ understanding of the history, functioning, pdf/professionals/ped࿝intake࿝form. questions and concerns of the family pdf ADHD screening tools Vanderbilt rating forms Parent- and teacher-completed forms that 6–12 y www.brightfutures.org/mentalhealth/ help a clinician diagnosis ADHD and to pdf/professionals/bridges/adhd.pdf categorize the problem into 1 of its various subtypes AAP ADHD toolkit A comprehensive toolkit developed from 6–12 y www.aap.org evidence-based guidelines for the diagnosis and treatment of children with ADHD; this resource toolkit contains a wide array of screening, diagnosis, treatment, and support materials for clinicians and other health care professionals ADHD indicates attention-deficit/hyperactivity disorder.

an ongoing process. The child requires for cochlear implantation should be language pathology, and other inter- regular audiologic reevaluations to de- considered when there is limited re- vention personnel. termine if there is fluctuating or pro- sidual hearing or when progress with gressive hearing loss. Middle-ear mon- amplification is insufficient. Recom- Most providers of pediatric health itoring is also essential. Hearing aid mendations to the family regarding co- care realize the importance of refer- selection, fitting, verification, and vali- chlear implantation should be based ring to an otolaryngologist, an audiol- dation require ongoing and regular on a team evaluation that includes au- ogist, and a speech-language patholo- visits with the audiologist. Candidacy diology, otology, psychology, speech- gist. Less recognized is the potential

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TABLE 8 Guidelines for Children with Abnormal Speech Development education, level of income, availability of Age, mo Referral Guidelines for Children With “Speech” Delay local resources, language in the home, 12 No differentiated babbling or vocal imitation and more. The role of the hearing health 18 No use of single words care team is to assist families in identi- 24 Single-word vocabulary of Յ10 words 30 Fewer than 100 words; no evidence of 2-word combinations; unintelligible fying all the options available to them 36 Fewer than 200 words; no use of telegraphic sentences; clarity Ͻ50% and to support them throughout the on- 48 Fewer than 600 words; no use of single sentences; clarity Յ80% going decision-making processes that Source: Matkin ND. Pediatr Rev. 1984;6:151. will occur throughout the child’s devel- opment. All members of the hearing TABLE 9 Guidelines for Children With Suspected Hearing Loss health care team, in conjunction with Age, Normal Development parents and on the basis of informed mo choice, should recognize that no deci- 0–4 Should startle to loud sounds, quiet to mother’s voice, momentarily cease activity when sion regarding intervention is “final,” sound is presented at a conversational level and periodic opportunities should be 5–6 Should correctly localize to sound presented in a horizontal plane, begin to imitate sounds in own speech repertoire or at least reciprocally vocalize with an adult identified for discussion regarding 7–12 Should correctly localize to sound presented in any plane, should respond to name, even progress, alternative interventions, and when spoken quietly new developments. 13–15 Should point toward an unexpected sound or to familiar objects or persons when asked 16–18 Should follow simple directions without gesture or other visual cues; can be trained to reach Medical follow-up includes ongoing toward an interesting toy at midline when a sound is presented evaluation and management of the ad- 19–24 Should point to body parts when asked; by 21 mo, can be trained to perform play audiometry equacy of hearing rehabilitation; ob- Source: Matkin ND. Pediatr Rev. 1984;6:151. servation for potential complications of hearing rehabilitation, such as otitis externa and cerumen impactions; and benefit of genetic and ophthalmologic occasionally, evaluation for other met- monitoring for appropriate speech evaluation of children and infants with abolic defects. Evaluation by a geneti- and language development. sensorineural hearing loss.17 cist and genetic testing can be impor- Speech and language evaluation by a Especially in young infants and chil- tant for diagnosis as well as for speech-language pathologist with dren, referral to an otolaryngologist providing the family with information training in working with children with for a complete diagnostic assessment for future planning purposes. hearing loss is also important for doc- is essential. More than 90% of the chil- The hearing health care team (com- umentation of baseline speech and dren with permanent hearing loss are prising the audiologist, otolaryngolo- language skills and implementing a born to “hearing” parents, resulting in gist, teachers of the child with hearing program of intervention that reflects a sense of urgency for parents of impairment, speech-language patholo- the family’s choice regarding language newly identified children with hearing gists, and other educational and med- development. loss to seek out answers regarding eti- ical personnel) should assist the fam- ology, risk of progression, implica- ily with intervention for hearing loss. At least one third of children with hear- tions, and other questions. If children Interventions may include observation ing loss will have an additional coexist- are indeed identified with sensorineu- with increased attention to speech and ing condition.3 Because many causes ral hearing loss, a variety of diagnostic language development, hearing aids, of hearing loss are associated with ab- tests can be recommended depending auditory-assisted systems for the school normal ophthalmologic findings, formal on the patient’s history and physical environment, or more invasive surgical ophthalmologic evaluation is appropri- examination. Otolaryngologists may hearing devices such as cochlear im- ate, not only to assist with the diagnosis play a role in diagnosis and treating plants or bone-anchored hearing aids. but also to optimize vision. A diagnosis of middle-ear fluid or other middle-ear The goal is to provide families with ap- Usher syndrome with associated pro- disorders as well as assisting in the propriate options so that they may make gressive hearing and vision loss may in- definitive diagnosis of the cause of well-informed decisions. Interventions fluence communication choices. sensorineural hearing loss.18 Diagnos- should be driven by family desires and Children with hearing loss should tic testing may include imaging of the guided by accurate and timely informa- also be monitored for developmental temporal bone to identify structural tion from all hearing-related health care and behavioral problems (attention- defects; genetic tests, such as for ab- professionals. Family goals and expecta- deficit/hyperactivity disorder, autism, normalities of the Connexin gene; and, tions are influenced by culture, parental learning disabilities) and referred for

PEDIATRICS Volume 124, Number 4, October 2009 1261 Downloaded from www.aappublications.org/news by guest on September 29, 2021 additional evaluation when necessary. sources available in their community for James J. Laughlin, MD Health care professionals can use children with hearing loss and should Andrea J. Leeds, MD screening tools to evaluate young chil- advocate for increasing options and LIAISONS dren periodically for such concerns choices for families. Otolaryngologists, Herschel R. Lessin, MD – Section on (Table 7) and refer for additional eval- audiologists, and speech-language pa- Administration and Practice Management uation when concerns arise. thologists with special training and ex- Olivia Whyte Thomas, MD – National Medical Association A medical professional should partici- perience in treating children should be pate as an active member of a family’s consulted for specific diagnosis, coun- STAFF seling, and treatment. Pediatric health Heather Fitzpatrick, MPH hearing health care team after diagno- Jennifer Mansour sis and provide input to assist in the care professionals should collaborate adequacy of the rehabilitative efforts to refer the child for comprehensive SECTION ON OTOLARYNGOLOGY–HEAD to monitor the child for progression educational counseling and treatment AND NECK SURGERY, 2007–2008 services. Communication among pro- David H. Darrow, MD, DDS, Chairperson and additional disabilities. Charles Bower, MD fessionals caring for a child with hear- Sukgi Susan Choi, MD HEARING REFERRAL RESOURCES ing loss is essential to ensure appro- Ellen Sue Deutsch, MD Key point 9: Abnormal hearing test re- priate case management. Scott R. Schoem, MD Mark S. Volk, MD, DMD sults require intervention and clinically appropriate referral, including otolaryn- COMMITTEE ON PRACTICE AND STAFF gology, audiology, speech-language pa- AMBULATORY MEDICINE, 2007–2008 Aleksandra Stolic, MPH thology, genetics, and early intervention. Robert D. Walker, MD, Chairperson Edward S. Curry, MD CONTRIBUTOR Pediatric health care professionals Lawrence D. Hammer, MD Christoph U. Lehmann, MD – Partnership for should maintain a list of referral re- Allen D. “Buz” Harlor Jr, MD Policy Implementation

REFERENCES 1. American Academy of Pediatrics, Committee on Practice and Ambulatory Medicine and Bright Futures Steering Committee. Recommendations for preventive pediatric health care. Pediatrics. 2007;120(6):1376 2. Yoshinaga-Itano C, Sedey AL, Coulter DK, Mehl AL. Language of early and later-identified children with hearing loss. Pediatrics. 1998;102(5):1161–1171 3. American Academy of Pediatrics, Joint Committee on Infant Hearing. Year 2007 position statement: principles and guidelines for early hearing detection and intervention programs. Pediatrics. 2007;120(4):898–921 4. American Academy of Pediatrics, Task Force on Newborn and Infant Hearing. Newborn and infant hearing loss: detection and intervention. Pediatrics. 1999;103(2):527–530 5. Bachmann KR, Arvedson JC. Early identification and intervention for children who are hearing impaired. Pediatr Rev. 1998;19(5):155–165 6. Berg AL, Spitzer JB, Garvin JH Jr. Ototoxic impact of cisplatin in pediatric oncology patients. Laryngoscope. 1999;109(11):1806–1814 7. Brookhouser PE, Worthington DW, Kelly WJ. Noise-induced hearing loss in children. Laryngoscope. 1992;102(6):645–655 8. Watkin PM, Baldwin M, McEnery G. Neonatal at risk screening and the identification of deafness. Arch Dis Child. 1991;66(10 spec No):1130–1135 9. Kittrell AP, Arjmand EM. The age of diagnosis of sensorineural hearing impairment in children. Int J Pediatr Otorhinolaryngol. 1997;40(2–3):97–106 10. Watkin PM, Baldwin M, Laoide S, Parental suspicion and identification of hearing impairment. Arch Dis Child. 1990;65(8):846–850 11. Coplan J. Deafness: ever heard of it? Delayed recognition of permanent hearing loss. Pediatrics. 1987;79(2):206–213 12. Grundfast KM, Lalwani AK. Practical approach to diagnosis and management of hereditary hear- ing impairment (HHI). Ear Nose Throat J. 1992;71(10):479–493 13. American Academy of Pediatrics, Otitis Media Guideline Panel. Managing otitis media with effusion in young children. Pediatrics. 1994;94(5):766–773 14. American Academy of Pediatrics, Subcommittee on Otitis Media With Effusion; American Academy of Family Physicians; American Academy of Otolaryngology–Head and Neck Surgery. Clinical prac- tice guideline: otitis media with effusion. Pediatrics. 2004;113(5):1412–1429

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15. Boone RT, Bower CM, Martin PF. Failed newborn hearing screens as presentation for otitis media with effusion in the newborn population. Int J Pediatr Otorhinolaryngol. 2005;69(3):393–397 16. Tharpe AM, Bess FH. Identification and management of children with minimal hearing loss. Int J Pediatr Otorhinolaryngol. 1991;21(1):41–45 17. Moeller MP, White KR, Shisler L. Primary care physicians’ knowledge, attitudes, and practices related to newborn hearing screening. Pediatrics. 2006;118(4):1357–1370 18. Strasnick B, Hoffman KK, Battista RA. Inner ear, genetic sensorineural hearing loss. eMedicine. Available at: www.emedicine.com/ent/topic223.htm. Accessed February 6, 2009 19. American Academy of Pediatrics, Subcommittee on Hyperbilirubinemia. Management of hyperbi- lirubinemia in the newborn infant 35 or more weeks of gestation [published correction appears in Pediatrics. 2004;114(4):1138]. Pediatrics. 2004;114(1):297–316

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