It is important to identify newborns with hearing loss by age 3 months so that intervention can begin by age 6 months. However, not all hearing impairment is present at birth and all infants and children should receive ongoing surveillance of hearing and communication skills at health supervision visits.1
This article will review the causes and consequences of hearing loss in childhood and describe how office-based technologies should be used to identify the hearing-impaired child. In addition, I will offer recommendations regarding the need to expedite evaluations by pediatric audiologists so that hearing-impaired children can be identified and treated in a timely fashion.
Overview of hearing loss in childhood
Childhood hearing loss is divided into 2 general categories, congenital and acquired (Table 1).
Congenital hearing loss is genetic in roughly 50% of cases. Approximately 70% to 80% of genetic hearing loss is autosomal recessive, 15% to 20% is autosomal dominant, and 2% is X-linked or mitochondrial. Fifteen percent of genetic hearing loss is part of a syndrome, such as Usher syndrome and Waardenburg syndrome, and more than 400 syndromes are associated with hearing loss.1 Nongenetic causes include prematurity, central nervous system injury, drug/alcohol use by mothers during pregnancy, and those caused by congenital infections.2
Acquired hearing loss is a consequence of various circumstances that can affect children, including perforated eardrums, protracted otitis media, otosclerosis, and cholesteatomas. Additional causes include prolonged and repetitive exposure to loud noises, exposure to ototoxic medications, infections, and head injury.1
Another way to view childhood hearing loss is to consider the anatomy involved. Conductive hearing loss involves problems between the external auditory canal and the cochlea, including vernix in newborns, cerumen in children, as well as aural atresia and otitis media. Sensory hearing loss is a consequence of cochlear dysfunction that may result from genetic causes, or exposure to ototoxic medications or infections. Neural causes of hearing loss include failure of function of the auditory nerve or disruption of auditory processing in the brain. Causes may include tumors, bleeding (intrapartum, postpartum or central nervous system [CNS] injury in childhood), infections, and auditory neuropathy.2
1. Joint Committee on Infant Hearing. Year 2019 position statement: principles and guidelines for early hearing detection and intervention programs. JEHDI. 2019;4(2):1-44.
2. Todd NW. The etiologies of childhood hearing impairment. In: NCHAM E-Book: A Resource Guide for Early Hearing Detection and Intervention. National Center for Hearing Assessment and Management; 2015; ch 6. Accessed April 8, 2020. http://www.infanthearing.org/ehdi-ebook/2015_ebook/6-Chapter6Etiologies2015.pdf
3. Centers for Disease Control and Prevention. 2017 Annual Data Early Hearing Detection and Intervention (EHDI) Program. Last reviewed December 5, 2019. Accessed April 8, 2020. https://www.cdc.gov/ncbddd/hearingloss/ehdi-data2017.html
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6. Barret TS, White KR. Trends in hearing loss among adolescents. Pediatrics. 2017;140(6):e20170619.
7. Robinshaw HM. Early intervention for hearing impairment: differences in the timing of communicative and linguistic development. Br J Audiol. 1995;29(6):315-334.
8. Apuzzo ML, Yoshinaga-Itano C. Early identification of infants with significant hearing loss and the Minnesota Child Development Inventory. Semin Hear. 1995;16:124-139.
9. Hagan JF, Shaw JS, Duncan PM, eds. Bright Futures: Guidelines for Health Supervision of Infants, Children and Adolescents. 4th ed. Elk Grove Village, IL: American Academy of Pediatrics; 2017.
10. Lieberthal AS, Carroll AE, Chonmaitree T, et al. The diagnosis and management of acute otitis media. Pediatrics. 2013;131:e964-e999.
11. Sabo DL. Assessment of the young pediatric patient. In: The NCHAM E-Book: A Resource Guide for Early Hearing Detection and Intervention. National Center for Hearing Assessment and Management; 2015:ch 5. Accessed April 8, 2020. https://www.infanthearing.org/ehdi-ebook/2015_ebook/5-Chapter5Assessment2015.pdf