With newborn hearing screening mandated in all states, the pediatrician has seen a profound reduction in the age when hearing loss is identified and advances in treatment that now allow treatment at very early ages.
With newborn hearing screening mandated in all states, the pediatrician has seen a profound reduction in the age when hearing loss is identified and advances in treatment now allow treatment at very early ages. Pediatricians who have been in practice for some time will remember not detecting hearing loss until there was a failure to develop language at age 2 to 3 years in patients with severe hearing loss. In patients with milder forms of hearing loss, the age was often much later.
This hearing screening is very good, but it is not perfect and will miss a number of cases. The pediatrician needs to be aware not only of always screening in the newborn, but also of indications for referral and workup as children get older. This article focuses on screening for hearing loss, risk factors for hearing loss, the physical exam, and workup of hearing loss as well as a brief discussion of treatment.
The American Academy of Pediatrics (AAP) recommends pediatricians follow the early hearing detection and intervention (EHDI) 1-3-6 plan that outlines what hearing assessments and referrals need to be completed before age 6 months. In brief, the EHDI 1-3-6 plan recommends currently mandated hospital-based screening and identification of a medical home for each infant. Infants who pass hospital-based screening will go into routine developmental and hearing-based screening in the pediatrician’s office.1,2
In terms of ongoing care for all infants, the pediatrician should:
· Provide parents with information on hearing, speech, and language milestones, with instruction to notify the pediatrician with any concerns.
· Identify and treat any middle-ear disease.
· Provide appropriate vision, hearing, and developmental screening.
· Refer for audiologic evaluation for any parental concern related to speech, hearing, or language development.
· Refer any high-risk child for audiologic evaluation at least once before age 30 months:
o Family history of hearing loss
o NICU (neonatal intensive care unit) stay of more than 5 days
o TORCH (toxoplasmosis, other infections [syphilis, varicella-zoster, parvovirus B19], rubella, cytomegalovirus [CMV], herpesvirus) infections
o Infections associated with hearing loss such as bacterial or viral meningitis
o Craniofacial anomalies
o Suggestion of a syndrome associated with hearing loss (eg, Alport syndrome)
o Neurodegenerative disorders (eg, Hunter syndrome) or sensory motor neuropathies (eg, Friedreich ataxia)
o Head trauma
Any patient who failed screening, had an incomplete screening, or needs rescreening should be referred for otoacoustic emissions (OAE) or auditory brainstem response (ABR) testing by age 1 month. Passing at this stage would place the infant into normal developmental screening.
If the infant fails at the 1-month screening, referral for a diagnostic evaluation should be made to a center capable of performing pediatric audiologic assessment with OAE, ABR, frequency-specific tone bursts, air/bone conduction, and the ability to sedate an infant in some cases. This assessment should be completed by age 3 months, and referral for intervention services should be made before age 6 months. Referral should include an otolaryngologist at a minimum and strong consideration for referral to an ophthalmologist because ophthalmologic abnormalities are increased among children with hearing loss.3 Additional referrals to a geneticist, developmental pediatrics, and other subspecialists (eg, cardiologist, nephrologist) as appropriate should be considered.
The pediatrician will need to be able to screen for hearing loss beyond the newborn period to identify acquired hearing loss (eg, meningitis, noise exposure), progressive or delayed hearing loss (eg, neurodegenerative syndromes, intrauterine infections), or hearing loss not identified on newborn screens. In addition to the monitoring of children with the previously mentioned risk factors, use of digital music players is another risk factor that should be considered.4
Older children and adolescents who may need a formal hearing screen may be identified by asking screening questions. Anyone with a positive response to any of the following questions adapted from the National Institute on Deafness and Other Communication Disorders (www.nidcd.nih.gov/health/hearing/pages/10ways.aspx) should trigger a more formal hearing evaluation:
· I hear but don’t always understand what others are saying.
· I have trouble hearing when others speak softly.
· I have trouble understanding someone if they are speaking in a different room.
· I have trouble hearing others when I am at a restaurant.
· I have trouble hearing others speak when they are not facing me.
· People tell me to turn down the TV.
· I find it hard to hear when I am in a group setting.
· I have difficulty catching most of the words when I go see a play or movie.
· I have trouble hearing on the telephone.
· I have trouble making out the words in songs.
One note of caution, however. A 2014 study utilizing the 10 hearing screening questions from the AAP’s Bright Futures program failed to identify adolescents at risk for hearing loss. The subjective assessment seemed to miss students with higher-frequency hearing loss, and the authors concluded that an objective screening measure may be preferred over the Bright Futures subjective assessment for routine preventive care.5
A complete discussion of audiology screening is beyond the scope of this article. Screening older children in the office, however, can be done with any of these tools: OAE, in-office audiology, and otoscope audiograms. Any patient with an abnormal result should be referred for formal audiologic evaluation.6
Perinatal history. TORCH infections are the classic diseases leading to hearing loss attributed to prenatal exposure. Only CMV, however, remains a substantial cause of hearing loss today.7 Prematurity is another important risk factor with particular attention to the infant’s course noting hypoxia, sepsis, hyperbilirubinemia, and exposure to potentially ototoxic antibiotics or diuretics.8
Family history. A family history of hearing loss at a young age raises suspicions for a genetic cause. Because many genetic causes are autosomal recessive in nature, many genetic causes of hearing present in families in whom no other child is impacted.8
Delays in milestones. Delays in motor milestones can be seen with cochleovestibular anomalies, vestibular disorders, some syndromic causes of hearing loss, and some acquired infectious causes of hearing loss such as meningitis.8
Infections and immunizations. It is important to remember that not all cases of meningitis are diagnosed. The pediatrician should be careful to look for hospitalizations where antibiotics were administered. Review of immunizations is also important because unimmunized children remain at increased risk of acute otitis media that can be complicated by meningitis.8
Noise exposure. Assessing high-risk activities is important in the older child or adolescent presenting for hearing loss. Particular attention should be paid to the child’s use of earphones and MP3 players because this risk is commonly underappreciated by parents.8
Physical examination of the child with hearing loss should include developmental assessment, measurement of growth parameters, and a general assessment focused on manifestations of syndromic hearing loss such as the examples listed in the Table.8 Particular focus on the head and neck exam is important. In a retrospective study of 114 children with hearing loss referred to a tertiary care center, head and neck abnormalities helped to establish the etiology of hearing loss in more than 40%-primarily craniofacial anomalies.9 Other abnormal physical findings of the head and neck to look for in patients with hearing loss include heterochromia of the irises; malformation of the auricle or ear canal; dimpling or skin tags around the auricle, cleft lip or palate; asymmetry or hypoplasia of the facial structures; and microcephaly.6
The preauricular area and outer ear should be examined for pits or sinuses, size and shape of the pinna, and patency of the external auditory canal. Significant cerumen should be removed because it also can lead to hearing loss. The tympanic membrane should be examined and pneumatic otoscopy performed. The tympanic membrane needs to be examined for perforation, scarring, and middle-ear fluid. Otitis media with effusion is a leading cause of acquired hearing loss, and acute otitis media may lead to short-term hearing loss.6,8
In older children and adolescents, hearing status can be assessed in the office using 256-Hz and 512-Hz tuning forks. These tools can also be used to perform the Weber and Rinne tests to help determine a conductive versus a sensorineural hearing loss (SNHL) etiology.8
Routine evaluation in a patient with unexplained SNHL has been questioned, citing low diagnostic yield.10 Laboratory testing, however, based on history, physical, and results of hearing testing, is a reasonable approach.
A urinalysis and electrocardiogram (ECG) on initial diagnosis of a new SNHL can detect several rare conditions. Proteinuria suggests a diagnosis of Alport syndrome, while the ECG may demonstrate a prolonged QT syndrome that might otherwise manifest with syncope or sudden cardiac death that is preventable with beta blocker therapy.11
Testing for congenital CMV infection and targeted genetic testing can identify the cause for hearing loss in many newborns.12 Testing for DNA mutations in the connexin 26 (Cx26) gene is reasonable because it is the most common genetic abnormality seen in newborns with congenital hearing loss.13 Genetic testing in SNHL is a rapidly evolving field. The development of microarray-based testing now allows for testing of multiple genes much like what is seen with newborn screening.14
Imaging is another consideration in patients with SNHL. Abnormalities on computed tomography and magnetic resonance imaging are reported in up to 40% of children with SNHL or mixed hearing loss, according to 1 series.10 Abnormalities help identify a cause of the hearing loss and help guide treatment. In some practices, ordering of imaging is deferred to the otolaryngologist because the most appropriate test depends on the particular situation and is in debate in the otolaryngology community.15,16
Treatment of hearing loss depends on etiology. Pressure equalization tubes for the tympanic membrane may improve hearing for children with middle-ear effusion, Eustachian tube dysfunction, or recurrent acute otitis media. Tumors and cholesteatoma require surgical excision or mastoidectomy. Some conditions that cause conductive hearing loss also can be treated with either surgery such as atresia or stenosis of the external auditory canal.
Hearing amplification is the next step in the treatment of hearing impairment. Although establishing an etiology is desirable, amplification before age 6 months is associated with improved outcomes and should not be delayed if an etiology is not yet established.17,18
Hearing aids will be recommended after an audiologic evaluation and based on child age, type of hearing loss, and patient family preference.19 Some patients who will not benefit from a standard hearing aid may benefit from a device that transmits sound through bone. These are available as an external device as well as a bone-anchored implantable hearing aid system. There also are systems now available that directly vibrate the ossicles in the middle ear.20,21
Cochlear implants are recommended for severe-to-profound bilateral SNHL in cases in which hearing aids have not been benficial.22,23 Bilateral cochlear implants have been advocated to improve functional hearing and performance in settings with a lot of background noise and increase ability to localize sound.24,25
Hearing loss today is a very treatable problem. The pediatrician needs to understand the importance of newborn hearing screening and how to manage abnormal results. Similarly, the pediatrician needs to understand when and why referral may be needed, how to work up a complaint of hearing loss, and the importance and timing of referral.
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17. Yoshinaga-Itano C. Efficacy of early identification and early intervention. Semin Hear. 2008;16(2):115-122.
18. 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.
19. Winter M, Eisenberg L. Amplification for infants: selection and verification. Otolaryngol Clin North Am. 1999;32(6):1051-1065.
20. Snik AF, Cremers CW. First audiometric results with the Vibrant soundbridge, a semi-implantable hearing device for sensorineural hearing loss. Audiology. 1999;38(6):335-338.
21. Chen DA, Backous DD, Arriaga MA, et al. Phase 1 clinical trial results of the Envoy System: a totally implantable middle ear device for sensorineural hearing loss. Otolaryngol Head Neck Surg. 2004;131(6):904-916.
22. Peters BR, Wyss J, Manrique M. Worldwide trends in bilateral cochlear implantation. Laryngoscope. 2010;120(suppl 2):S17-S44.
23. Cochlear implants in adults and children: summary of the NIH consensus. Office of Medical Applications of Research, National Institutes of Health. Aust N Z J Surg. 1997;67(6):379-380.
24. Balkany T, Hodges A, Telischi F, et al. William House Cochlear Implant Study Group: position statement on bilateral cochlear implantation. Otol Neurotol. 2008;29(2):107-108.
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Dr Bass is chief medical information officer and associate professor of medicine and of pediatrics, Louisiana State University Health Sciences Center–Shreveport. The author has nothing to disclose in regard to affiliations with or financial interests in any organizations that may have an interest in any part of this article.