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Hearing impairment in children: Etiology

Richard JH Smith, MD
Adrian Gooi, MD, FRCS(C)
Section Editor
Glenn C Isaacson, MD, FAAP
Deputy Editor
Carrie Armsby, MD, MPH


Hearing loss in the first years of life can cause delays in speech, language, and cognitive development [1]. Speech and language delays secondary to hearing loss are often preventable [2,3]. Thus, early identification of hearing impairment, whether it is permanent (usually sensorineural) or temporary (usually conductive), is the key to a child's success with communication [4-7].

Significant hearing loss occurs in 1 to 2 per 1000 newborns and in 2 per 1000 young children. However, nearly all children develop transient hearing loss related to middle ear infections during the period from birth to 11 years of age [8]. Knowledge of the etiology of the hearing loss, particularly if genetic, can affect family planning and assist in the development of the optimal habilitation plan for the patient [9,10].

The anatomy and physiology of the ear and the etiology of hearing impairment in children are reviewed here. The evaluation and treatment of hearing impairment are discussed separately. (See "Hearing impairment in children: Evaluation" and "Hearing impairment in children: Treatment".)


The ear is divided into three anatomic segments (figure 1):

The outer ear, comprising the auricle and external auditory canal (EAC)

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Literature review current through: Nov 2017. | This topic last updated: Sep 05, 2017.
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  1. Kral A, O'Donoghue GM. Profound deafness in childhood. N Engl J Med 2010; 363:1438.
  2. Yoshinaga-Itano C, Sedey AL, Coulter DK, Mehl AL. Language of early- and later-identified children with hearing loss. Pediatrics 1998; 102:1161.
  3. Downs MP, Yoshinaga-Itano C. The efficacy of early identification and intervention for children with hearing impairment. Pediatr Clin North Am 1999; 46:79.
  4. Watkin P, McCann D, Law C, et al. Language ability in children with permanent hearing impairment: the influence of early management and family participation. Pediatrics 2007; 120:e694.
  5. Vohr B, Jodoin-Krauzyk J, Tucker R, et al. Early language outcomes of early-identified infants with permanent hearing loss at 12 to 16 months of age. Pediatrics 2008; 122:535.
  6. Pimperton H, Kennedy CR. The impact of early identification of permanent childhood hearing impairment on speech and language outcomes. Arch Dis Child 2012; 97:648.
  7. Ching TYC, Dillon H, Button L, et al. Age at Intervention for Permanent Hearing Loss and 5-Year Language Outcomes. Pediatrics 2017; 140.
  8. Northern JL, Downs MP. Hearing in children, 4th ed, Williams and Wilkins, Baltimore 1991.
  9. Erenberg A, Lemons J, Sia C, et al. Newborn and infant hearing loss: detection and intervention.American Academy of Pediatrics. Task Force on Newborn and Infant Hearing, 1998- 1999. Pediatrics 1999; 103:527.
  10. Roizen NJ. Etiology of hearing loss in children. Nongenetic causes. Pediatr Clin North Am 1999; 46:49.
  11. Stewart JM, Downs MP. Congenital conductive hearing loss: the need for early identification and intervention. Pediatrics 1993; 91:355.
  12. Jahrsdoerfer RA. Congenital atresia of the ear. Laryngoscope 1978; 88:1.
  13. Teele DW, Klein JO, Rosner B. Epidemiology of otitis media during the first seven years of life in children in greater Boston: a prospective, cohort study. J Infect Dis 1989; 160:83.
  14. Alper DM, Bluestone CD. Advanced therapy in otitis media, BC Decker Incorporated, Hamilton, Ontario 2004.
  15. Klein JO. Otitis media. Clin Infect Dis 1994; 19:823.
  16. Infante-Rivard C, Fernández A. Otitis media in children: frequency, risk factors, and research avenues. Epidemiol Rev 1993; 15:444.
  17. Bluestone CD, Klein JO. Intratemporal complications and sequelae of otitis media. In: Pediatric otolaryngology, Bluestone CD, Stool SE (Eds), WB Saunders, 1983. Vol 1.
  18. Klausen O, Møller P, Holmefjord A, et al. Lasting effects of otitis media with effusion on language skills and listening performance. Acta Otolaryngol Suppl 2000; 543:73.
  19. Petinou KC, Schwartz RG, Gravel JS, Raphael LJ. A preliminary account of phonological and morphophonological perception in young children with and without otitis media. Int J Lang Commun Disord 2001; 36:21.
  20. Bennett KE, Haggard MP, Silva PA, Stewart IA. Behaviour and developmental effects of otitis media with effusion into the teens. Arch Dis Child 2001; 85:91.
  21. Paradise JL, Feldman HM, Campbell TF, et al. Effect of early or delayed insertion of tympanostomy tubes for persistent otitis media on developmental outcomes at the age of three years. N Engl J Med 2001; 344:1179.
  22. Paradise JL, Feldman HM, Campbell TF, et al. Early versus delayed insertion of tympanostomy tubes for persistent otitis media: developmental outcomes at the age of three years in relation to prerandomization illness patterns and hearing levels. Pediatr Infect Dis J 2003; 22:309.
  23. Paradise JL, Dollaghan CA, Campbell TF, et al. Otitis media and tympanostomy tube insertion during the first three years of life: developmental outcomes at the age of four years. Pediatrics 2003; 112:265.
  24. Paradise JL, Campbell TF, Dollaghan CA, et al. Developmental outcomes after early or delayed insertion of tympanostomy tubes. N Engl J Med 2005; 353:576.
  25. Paradise JL, Feldman HM, Campbell TF, et al. Tympanostomy tubes and developmental outcomes at 9 to 11 years of age. N Engl J Med 2007; 356:248.
  26. Cannon CR, Jahrsdoerfer RA. Temporal bone fractures. Review of 90 cases. Arch Otolaryngol 1983; 109:285.
  27. Wasserman EE, Nelson K, Rose NR, et al. Maternal thyroid autoantibodies during the third trimester and hearing deficits in children: an epidemiologic assessment. Am J Epidemiol 2008; 167:701.
  28. Weller TH. The cytomegaloviruses: ubiquitous agents with protean clinical manifestations. I. N Engl J Med 1971; 285:203.
  29. Ogawa H, Suzutani T, Baba Y, et al. Etiology of severe sensorineural hearing loss in children: independent impact of congenital cytomegalovirus infection and GJB2 mutations. J Infect Dis 2007; 195:782.
  30. Kimani JW, Buchman CA, Booker JK, et al. Sensorineural hearing loss in a pediatric population: association of congenital cytomegalovirus infection with intracranial abnormalities. Arch Otolaryngol Head Neck Surg 2010; 136:999.
  31. Misono S, Sie KC, Weiss NS, et al. Congenital cytomegalovirus infection in pediatric hearing loss. Arch Otolaryngol Head Neck Surg 2011; 137:47.
  32. McGee T, Wolters C, Stein L, et al. Absence of sensorineural hearing loss in treated infants and children with congenital toxoplasmosis. Otolaryngol Head Neck Surg 1992; 106:75.
  33. Strasnick B, Jacobson JT. Teratogenic hearing loss. J Am Acad Audiol 1995; 6:28.
  34. Centers for Disease Control. Rubella prevention. MMWR 1990; 39(RR15):1.
  35. Stamos JK, Rowley AH. Timely diagnosis of congenital infections. Pediatr Clin North Am 1994; 41:1017.
  36. Wilkins A, Prabhu SP, Huang L, et al. Frequent association of cochlear nerve canal stenosis with pediatric sensorineural hearing loss. Arch Otolaryngol Head Neck Surg 2012; 138:383.
  37. Tomaski SM, Grundfast KM. A stepwise approach to the diagnosis and treatment of hereditary hearing loss. Pediatr Clin North Am 1999; 46:35.
  38. Cohn ES, Kelley PM, Fowler TW, et al. Clinical studies of families with hearing loss attributable to mutations in the connexin 26 gene (GJB2/DFNB1). Pediatrics 1999; 103:546.
  39. Parker M, Bitner-Glindzicz M. Genetic investigations in childhood deafness. Arch Dis Child 2015; 100:271.
  40. Grundfast KM, Atwood JL, Chuong D. Genetics and molecular biology of deafness. Otolaryngol Clin North Am 1999; 32:1067.
  41. Hereditary Hearing loss Homepage. Available at: http://hereditaryhearingloss.org/ (Accessed on September 20, 2016).
  42. Skvorak Giersch AB, Morton CC. Genetic causes of nonsyndromic hearing loss. Curr Opin Pediatr 1999; 11:551.
  43. Smith RJ, Van Camp G. Non-syndromic hearing impairment: gene linkage and cloning. Int J Pediatr Otorhinolaryngol 1999; 49 Suppl 1:S159.
  44. Zelante L, Gasparini P, Estivill X, et al. Connexin26 mutations associated with the most common form of non-syndromic neurosensory autosomal recessive deafness (DFNB1) in Mediterraneans. Hum Mol Genet 1997; 6:1605.
  45. del Castillo I, Villamar M, Moreno-Pelayo MA, et al. A deletion involving the connexin 30 gene in nonsyndromic hearing impairment. N Engl J Med 2002; 346:243.
  46. Schraders M, Oostrik J, Huygen PL, et al. Mutations in PTPRQ are a cause of autosomal-recessive nonsyndromic hearing impairment DFNB84 and associated with vestibular dysfunction. Am J Hum Genet 2010; 86:604.
  47. Mochizuki T, Lemmink HH, Mariyama M, et al. Identification of mutations in the alpha 3(IV) and alpha 4(IV) collagen genes in autosomal recessive Alport syndrome. Nat Genet 1994; 8:77.
  48. Mhatre AN, Lalwani AK. Molecular genetics of deafness. Otolaryngol Clin North Am 1996; 29:421.
  49. Kenna MA, Wu BL, Cotanche DA, et al. Connexin 26 studies in patients with sensorineural hearing loss. Arch Otolaryngol Head Neck Surg 2001; 127:1037.
  50. Frei K, Ramsebner R, Lucas T, et al. GJB2 mutations in hearing impairment: identification of a broad clinical spectrum for improved genetic counseling. Laryngoscope 2005; 115:461.
  51. Denoyelle F, Weil D, Maw MA, et al. Prelingual deafness: high prevalence of a 30delG mutation in the connexin 26 gene. Hum Mol Genet 1997; 6:2173.
  52. Rabionet R, Gasparini P, Estivill X. Connexins and deafness homepage. davinci.crg.es/deafness/ (Accessed on January 18, 2006).
  53. Duggal P, Vesely MR, Wattanasirichaigoon D, et al. Mutation of the gene for IsK associated with both Jervell and Lange-Nielsen and Romano-Ward forms of Long-QT syndrome. Circulation 1998; 97:142.
  54. Zarchi O, Attias J, Raveh E, et al. A comparative study of hearing loss in two microdeletion syndromes: velocardiofacial (22q11.2 deletion) and Williams (7q11.23 deletion) syndromes. J Pediatr 2011; 158:301.
  55. Pfister MH, Apaydin F, Turan O, et al. A second family with nonsyndromic sensorineural hearing loss linked to Xp21.2: refinement of the DFN4 locus within DMD. Genomics 1998; 53:377.
  56. Van Naarden K, Decouflé P. Relative and attributable risks for moderate to profound bilateral sensorineural hearing impairment associated with lower birth weight in children 3 to 10 years old. Pediatrics 1999; 104:905.
  57. van Dommelen P, Verkerk PH, van Straaten HL, Dutch Neonatal Intensive Care Unit Neonatal Hearing Screening Working Group. Hearing loss by week of gestation and birth weight in very preterm neonates. J Pediatr 2015; 166:840.
  58. Douek E, Dodson HC, Bannister LH, et al. Effects of incubator noise on the cochlea of the newborn. Lancet 1976; 2:1110.
  59. Razi MS, Das VK. Effects of adverse perinatal events on hearing. Int J Pediatr Otorhinolaryngol 1994; 30:29.
  60. Cristobal R, Oghalai JS. Hearing loss in children with very low birth weight: current review of epidemiology and pathophysiology. Arch Dis Child Fetal Neonatal Ed 2008; 93:F462.
  61. Ehrenhaft PM, Wagner JL, Herdman RC. Changing prognosis for very low birth weight infants. Obstet Gynecol 1989; 74:528.
  62. Mervis CA, Decouflé P, Murphy CC, Yeargin-Allsopp M. Low birthweight and the risk for mental retardation later in childhood. Paediatr Perinat Epidemiol 1995; 9:455.
  63. Wickremasinghe AC, Risley RJ, Kuzniewicz MW, et al. Risk of Sensorineural Hearing Loss and Bilirubin Exchange Transfusion Thresholds. Pediatrics 2015; 136:505.
  64. Amin SB, Saluja S, Saili A, et al. Auditory toxicity in late preterm and term neonates with severe jaundice. Dev Med Child Neurol 2017; 59:297.
  65. de Vries LS, Lary S, Dubowitz LM. Relationship of serum bilirubin levels to ototoxicity and deafness in high-risk low-birth-weight infants. Pediatrics 1985; 76:351.
  66. Fortnum HM. Hearing impairment after bacterial meningitis: a review. Arch Dis Child 1992; 67:1128.
  67. Fortnum H, Davis A. Epidemiology of permanent childhood hearing impairment in Trent Region, 1985-1993. Br J Audiol 1997; 31:409.
  68. Dodge PR, Davis H, Feigin RD, et al. Prospective evaluation of hearing impairment as a sequela of acute bacterial meningitis. N Engl J Med 1984; 311:869.
  69. Richardson MP, Reid A, Tarlow MJ, Rudd PT. Hearing loss during bacterial meningitis. Arch Dis Child 1997; 76:134.
  70. Kaplan SL, Catlin FI, Weaver T, Feigin RD. Onset of hearing loss in children with bacterial meningitis. Pediatrics 1984; 73:575.
  71. Vienny H, Despland PA, Lütschg J, et al. Early diagnosis and evolution of deafness in childhood bacterial meningitis: a study using brainstem auditory evoked potentials. Pediatrics 1984; 73:579.
  72. Kaplan SL, Woods CR. Neurologic complications of bacterial meningitis in children. Curr Clin Top Infect Dis 1992; 12:37.
  73. Kopelovich JC, Germiller JA, Laury AM, et al. Early prediction of postmeningitic hearing loss in children using magnetic resonance imaging. Arch Otolaryngol Head Neck Surg 2011; 137:441.
  74. Bendush CL. Ototoxicity: Clinical considerations and comparative information. In: The aminoglycosides: Microbiology, clinical use and toxicology, Marcel Dekker, New York 1982. p.453.
  75. Robertson CM, Tyebkhan JM, Peliowski A, et al. Ototoxic drugs and sensorineural hearing loss following severe neonatal respiratory failure. Acta Paediatr 2006; 95:214.
  76. Prezant TR, Agapian JV, Bohlman MC, et al. Mitochondrial ribosomal RNA mutation associated with both antibiotic-induced and non-syndromic deafness. Nat Genet 1993; 4:289.
  77. Casano RA, Johnson DF, Bykhovskaya Y, et al. Inherited susceptibility to aminoglycoside ototoxicity: genetic heterogeneity and clinical implications. Am J Otolaryngol 1999; 20:151.
  78. Stavroulaki P, Apostolopoulos N, Dinopoulou D, et al. Otoacoustic emissions--an approach for monitoring aminoglycoside induced ototoxicity in children. Int J Pediatr Otorhinolaryngol 1999; 50:177.
  79. Gerber SE. The handbook of pediatric audiology, Gallaudet University Press, Washington, DC 1996.
  80. Berg AL, Spitzer JB, Garvin JH Jr. Ototoxic impact of cisplatin in pediatric oncology patients. Laryngoscope 1999; 109:1806.
  81. Gallagher KL, Jones JK. Furosemide-induced ototoxicity. Ann Intern Med 1979; 91:744.
  82. Smith RJ, Bale JF Jr, White KR. Sensorineural hearing loss in children. Lancet 2005; 365:879.
  83. Niskar AS, Kieszak SM, Holmes AE, et al. Estimated prevalence of noise-induced hearing threshold shifts among children 6 to 19 years of age: the Third National Health and Nutrition Examination Survey, 1988-1994, United States. Pediatrics 2001; 108:40.
  84. Prasher D. New strategies for prevention and treatment of noise-induced hearing loss. Lancet 1998; 352:1240.
  85. Shih L. Cochlear hearing loss in neurotology. In: Neurotology, Jackler R, Brackman DE (Eds), Mosby, St. Louis 1994. p.619.
  86. Recommendations for a noise standard. In: Occupational Noise Exposure: NIOSH Publication Number 98-126/CDC/NIOSH. www.cdc.gov/niosh/docs/98-126/chap1.html (Accessed on December 19, 2006).
  87. Fligor BJ, Cox LC. Output levels of commercially available portable compact disc players and the potential risk to hearing. Ear Hear 2004; 25:513.
  88. Portnuff C, Fligor B. "Sound output levels of the iPod and other MP3 players. Is there potential risk to hearing?" Presented at Noise-Induced Hearing Loss in Children at Work and Play Conference, Cincinnati, OH, October 19-20, 2006. www.hearingconservation.org/docs/virtualPressRoom/portnuff.htm (Accessed on December 19, 2006).
  89. Jackler RK. Acoustic neuroma. In: Neurotology, Jackler R, Brackman DE (Eds), Mosby, St. Louis 1994. p.729.
  90. Prasher D. Heavy metals and noise exposure: health effects. Noise Health 2009; 11:141.
  91. Shargorodsky J, Curhan SG, Henderson E, et al. Heavy metals exposure and hearing loss in US adolescents. Arch Otolaryngol Head Neck Surg 2011; 137:1183.
  92. Hall JW III, Mueller HG III. Audiologists' desk reference, Singular Publishing Group, San Diego 1997. Vol 1.
  93. Jerger J, Musiek F. Report of the Consensus Conference on the Diagnosis of Auditory Processing Disorders in School-Aged Children. J Am Acad Audiol 2000; 11:467.
  94. Varga R, Kelley PM, Keats BJ, et al. Non-syndromic recessive auditory neuropathy is the result of mutations in the otoferlin (OTOF) gene. J Med Genet 2003; 40:45.
  95. Deltenre P, Mansbach AL, Bozet C, et al. Auditory neuropathy with preserved cochlear microphonics and secondary loss of otoacoustic emissions. Audiology 1999; 38:187.