Impact of Neurodevelopmental Disorders on Hearing in Children and Adolescents

Chapter

Abstract

Recent advances in maternal–fetal medicine and neonatology have led to unprecedented increase in the survival of severely preterm babies and babies with severe neurodevelopmental disabilities. These babies typically present with multiple neurosensory impairments and pose a significant challenge to neurodiagnosis and intervention. This review will describe some common neurodevelopmental disorders that impact the auditory system and present case studies to highlight the current technologies available to diagnose and treat the hearing problems.

References

  1. 1.
    Committee on fetus and newborn. Joint statement on neonatal screening for hearing impairment. Pediatrics. 1971;47(6):1085.Google Scholar
  2. 2.
    American academy of pediatrics task force on newborn and infant hearing. Newborn and infant hearing loss: detection and intervention. Pediatrics. 1999;103:527–29.CrossRefGoogle Scholar
  3. 3.
    Yoshinaga-Itano C, Sedey AL, Coulter DK, Mehl AL. Language of early- and later-identified children with hearing loss. Pediatrics. 1998;102(5):1161–71.PubMedCrossRefGoogle Scholar
  4. 4.
    Downs MP, Yoshinaga-Itano C. The efficacy of early identification and intervention for children with hearing impairment. Pediatr Clin North Am. 1999;46(1):79–87.PubMedCrossRefGoogle Scholar
  5. 5.
    Yoshinaga-Itano C. Early intervention after universal neonatal hearing screening: impact on outcomes. Ment Retard Dev Disabil Res Rev. 2003;9(4):252–66.PubMedCrossRefGoogle Scholar
  6. 6.
    Guidelines development conference on the identification and management of infants with auditory neuropathy, International Newborn Hearing Screening Conference, Como, Italy, June 19-21, 2008.Google Scholar
  7. 7.
    Rance G, Barker EJ, Sarant JZ, Ching TY. Receptive language and speech production in children with auditory neuropathy/dys-synchrony type hearing loss. Ear Hear. 2007;25:34–46.CrossRefGoogle Scholar
  8. 8.
    Marlin S, Feldmann D, Nyugen Y, Rouillon I, Loundon N, Jonard L, Bonnet C, Couderc R, Garabedian EN, Petit C, Denoyelle F. Temperature-sensitive auditory neuropathy associated with an otoferlin mutation: deafening fever! Biochem Biophys Res Commun. 2010;394(3):737–42.PubMedCrossRefGoogle Scholar
  9. 9.
    Berlin CI, Hood LJ, Goforth-Barter L, Bordelon J. Auditory neuropathy: three time courses after early identification. ARO abstracts. 1999;22:169.Google Scholar
  10. 10.
    Starr A, Sininger Y, Winter M, Derebery MJ, Oba S, Michalewski HJ. Transient deafness due to temperature-sensitive auditory neuropathy. Ear Hear. 1998;19(3):169–79.PubMedCrossRefGoogle Scholar
  11. 11.
    Foerst A, Beutner D, Lang-Roth R, Huttenbrink KB, von Wedel H, Walger M. Prevalence of auditory neuropathy/synpatopathy in a population of children with profound hearing loss. Int J Pediatr Otorhinolaryngol. 2006;70(8):1415–22.PubMedCrossRefGoogle Scholar
  12. 12.
    Berlin CI, Hood LJ, Morlet T, Den Z, Goforth L. The search for auditory neuropathy patients and connexin 26 patients in schools for the deaf. ARO abstracts. 2000;23:23.Google Scholar
  13. 13.
    Lee JSM, McPherson B, Yuen KCP, Wong LLN. Screening for auditory neuropathy in a school for hearing impaired children. Int J Pediatr Otorhinolaryngol. 2001;61:39–46.PubMedCrossRefGoogle Scholar
  14. 14.
    Cheng X, Li L, Brashears S, Morlet T, Ng SS, Berlin C, Hood L, Keats B. Connexin 26 variants and auditory neuropathy/dys-synchrony among children in school for the deaf. Am J Med Genet. 2005;139(1):13–18.PubMedCrossRefGoogle Scholar
  15. 15.
    Sininger YS. Identification of auditory neuropathy in infants and children. Sem Hear. 2002;23:193–200.CrossRefGoogle Scholar
  16. 16.
    Rea PA, Gibson WPR. Evidence for surviving outer hair cell function in congenitally deaf ears. Laryngoscope. 2003;113:230–34.Google Scholar
  17. 17.
    Rance G, Beer DE, Cone-Wesson B, Shepherd RK, Dowell RC, King AM, Rickards FW, Clark GM. Clinical findings for a group of infants and young children with auditory neuropathy. Ear Hear. 1999;20(3):238–52.PubMedCrossRefGoogle Scholar
  18. 18.
    Sininger YS, Oba S. Patients with auditory neuropathy: who are they and what can they hear? In: Sininger Y, Starr A, editors. Auditory neuropathy: new perspectives in hearing disorders, San Diego: Singular-Thomson Learning; 2001. pp. 15–36.Google Scholar
  19. 19.
    Madden C, Rutter M, Hilbert L, Greinwald JH, Choo DI. Clinical and audiological features in auditory neuropathy. Arch Otolaryngol Head Neck Surg. 2002;128(9):1026–30.PubMedGoogle Scholar
  20. 20.
    Starr A, Picton TW, Sininger Y, Hood LJ, Berlin CI. Auditory neuropathy. Brain. 1996;119 3:741–53.PubMedCrossRefGoogle Scholar
  21. 21.
    Oysu C, Aslan I, Basaran B, Baserer N. The site of hearing loss in Refsum’s disease. Int J Pediatr Otolrhinolaryngol. 2001;16:129–34.CrossRefGoogle Scholar
  22. 22.
    Jutras B, Russell LJ, Hurteau AM, Chapdelaine M. Auditory neuropathy in siblings with Waardenburg’s syndrome. Int J Pediatr Otorhinolaryngol. 2003;67(10):1133–42.PubMedCrossRefGoogle Scholar
  23. 23.
    Corley VM, Crabbe LS. Auditory neuropathy and a mitochondrial disorder in a child: case study. J Am Acad Audiol. 1999;11:484–88.Google Scholar
  24. 24.
    Deltenre P, Mansbach AL, Bozet C, Clercx A, Hecox KE. Auditory neuropathy: a report on three cases with early onsets and major neonatal illnesses. Electroencephalogr Clin Neurophysiol. 1997;104(1):17–22.PubMedCrossRefGoogle Scholar
  25. 25.
    Teagle HFB, Roush PA, Woodard JS, Hatch DR, Zdanski CJ, Buss E, Buchman CA. Cochlear implantation in children with auditory neuropathy spectrum disorder. Ear Hear. 2010;31(3):325–35.PubMedCrossRefGoogle Scholar
  26. 26.
    Starr A, Isaacson B, Michaelewski H, Zeng F, Kong Y, Beale P, et al. A dominantly inherited progressive deafness affecting distal auditory nerve and hair cells. J Assoc Res Otolaryngol. 2004;5:411–26.PubMedCrossRefGoogle Scholar
  27. 27.
    Starr A, Picton TW, Kim R. Pathophysiology of auditory neuropathy. In: Sininger Y, Starr A, editors. Auditory neuropathy: new perspectives in hearing disorders. San Diego: Singular-Thomson Learning; 2001. pp. 67–82.Google Scholar
  28. 28.
    Hall III JW. ECochG: clinical applications and populations; Auditory Neuropathy. In: Hall III JW, editor. New handbook of auditory evoked responses. Boston, MA: Pearson Education; 2007. pp. 138–53.Google Scholar
  29. 29.
    Demmler GJ. Summary of a workshop on surveillance for congenital cytomegalovirus disease. Rev Infect Dis. 1991;13:315–29.PubMedCrossRefGoogle Scholar
  30. 30.
    Pass RF, Stagno S, Myers GJ. Outcome of symptomatic congenital cytomegalic infection: results of long term longitudinal follow-up. Pediatrics. 1980;66:758–62.PubMedGoogle Scholar
  31. 31.
    Ramsay ME, Miller R, Peckham CS. Outcome of confirmed symptomatic congenital cytomegalovirus infection. Arch Dis Child. 1991;66:1068–9.PubMedCrossRefGoogle Scholar
  32. 32.
    Bopanna SB, Pass RF, Brit WJ, Stagno S, Alford A. Symptomatic congenital cytomegalovirus infection: neonatal morbidity and mortality. Pediatr Infect Dis J. 1992;11:93–99.CrossRefGoogle Scholar
  33. 33.
    Noyola DE, Demmler GJ, Nelson CT, Griesser C, Wiliamson WD, Atkins JT, et al. Early predictors of neurodevelopmental outcome in symptomatic congenital cytomegalovirus infection. J Pediatr. 2001;138:325–31.PubMedCrossRefGoogle Scholar
  34. 34.
    Fowler KB, Bopanna SB. Congenital cytomegalovirus (CMV) infection and hearing deficit. J Clin Virol. 2006;35:226–31.PubMedCrossRefGoogle Scholar
  35. 35.
    Bradford RD, Cloud G, Lakeman AD, Bopanna S, Kimberlin DW, Jacobs R, et al. Detection of cytomegalovirus (CMV) DNA by polymerase chain reaction is associated with hearing loss in newborns with symptomatic congential CMV infection involving the central nervous system. J Infect Dis. 2005;191:227–33.PubMedCrossRefGoogle Scholar
  36. 36.
    Bopanna SB, Fowler KB, Pass RF, Rivera LB, Bradford RD, Lakeman FD, Britt WJ. Congenital cytomegalovirus infection: association between virus burden in infancy and hearing loss. J Pediatr. 2005;146:817–23.CrossRefGoogle Scholar
  37. 37.
    Noyola DE, Demmler GJ, Williamson WD, Greisser C, Sellers S, Llorente A, et al. Cytomegalovirus urinary excretion and long term outcome in children with congenital cytomegalovirus infection. Pediatr Infect Dis J. 2000;19:5050–10.Google Scholar
  38. 38.
    Myers EN, Stool S. Cytomegalic inclusion disease of the inner ear. Laryngoscope. 1968;78 11:1904–15.PubMedCrossRefGoogle Scholar
  39. 39.
    Davis LE, Johnsson LG, Korufeld M. Cytomegalovirus labyrinthitis in an infant: morphological, virological and immunofluorescent studies. J Neuropathol Exp Neurol. 1981;40:9–19.PubMedGoogle Scholar
  40. 40.
    Bachor EH, Sudhoff R, Litschel R, Karmody CS. The pathology of the temporal bones of a child with acquired cytomegalovirus infection: studies by light microscopy, immunohistochemistry and polymerase chain reaction. J Pediatr Otorhinolaryngol. 2000;55:215–24.CrossRefGoogle Scholar
  41. 41.
    Rarey KE, Davis LE. Temporal bone histopathology 14 years after cytomegalic inclusion disease: a case study. Laryngoscope. 1993;103:904–9.PubMedCrossRefGoogle Scholar
  42. 42.
    Sugiura S, Yoshikawa T, Nishiyama Y, Morishita Y, Sato E, Hattori T, Nakashima T. Detection of human cytomegalovirus DNA in perilymph of patients with sensorineural hearing loss using real-time PCR. J Med Virol. 2003;69:72–77.PubMedCrossRefGoogle Scholar
  43. 43.
    Bauer PW, Parizi-Robinson M, Roland PS, Yegappan S. Cytomegalovirus in the perilymphatic fluid. Laryngoscope. 2005;115:223–25.PubMedCrossRefGoogle Scholar
  44. 44.
    Nelson CT, Demmler GJ. Cytomegalovirus infection in the mother, fetus, and newborn infant. Clin Perinatol. 1997;24:151–60.PubMedGoogle Scholar
  45. 45.
    Kenneson A, Cannon MJ. Review and meta-analysis of the epidemiology of congenital cytomegalovirus (CMV) infection. Rev Med Virol. 2007;17:253–76.PubMedCrossRefGoogle Scholar
  46. 46.
    Adler SP, Nigro G, Pereira L. Recent advances in the prevention and treatment of congenital cytomegalovirus infections. Semin Perinatol. 2007;31:10–18.PubMedCrossRefGoogle Scholar
  47. 47.
    Bopanna SB, Rivera LB, Fowler KB, Mach M, Britt WJ. Intrauterine transmission of cytomegalovirus to infants of women with preconceptional immunity. N Engl J Med. 2001;344:1366–71.CrossRefGoogle Scholar
  48. 48.
    McCluskey RR, Sandin R, Greene J. Detection of airborne cytomegalovirus in hospital rooms of immunocompromised patients. J Virol Methods. 1996;56:115–18.PubMedCrossRefGoogle Scholar
  49. 49.
    Cannon MJ, Davis KF. Washing our hands of the congenital cytomegalovirus disease epidemic. BMC Public Health. 2005;5:70.PubMedCrossRefGoogle Scholar
  50. 50.
    Staras SA, Flanders WD, Dollard SC, Pass RF, McGowan JE Jr, Cannon MJ. Influence of sexual activity on cytomegalovirus seroprevalence in the United States, 1988-1994. Sex Transm Dis. 2008;35:472–79.PubMedCrossRefGoogle Scholar
  51. 51.
    Rivera LB, Bopanna SB, Fowler KB, Britt WJ, Stagno S, Pass RF. Predictors of hearing loss in children with symptomatic congenital cytomegalovirus infection. Pediatrics. 2000;110:762–67.CrossRefGoogle Scholar
  52. 52.
    Fowler K. Do perinatal factors predict hearing loss in children with asymptomatic congenital CMV infection? Am J Epidemiol. 2003;157:S84.Google Scholar
  53. 53.
    Barbi M, Binda S, Caroppo S, Ambrosetti U, Corbetta C, Sergi P. A wider role for congenital cytomegalovirus infection in sensorineural hearing loss. Pediatr Infect Dis J. 2003;22(1):39–42.PubMedCrossRefGoogle Scholar
  54. 54.
    Ciorba A, Bovo R, Trevisi P, Bianchini C, Arboretti R, Martini A. Rehabilitation and outcome of severe profound deafness in a group of 16 infants affected by congenital cytomegalovirus infection. Eur Arch Otorhinolaryngol. 2009;266:1539–46.PubMedCrossRefGoogle Scholar
  55. 55.
    Kimberlin DW, Lin C-Y, Sanchez PJ, Demmler GJ, Dankner DW, Shelton M, et al. Effect of ganciclovir therapy on hearing in symptomatic congenital cytomegalovirus disease involving the central nervous system: a randomized, controlled trial. J Pediatr. 2003;143:16–25.PubMedCrossRefGoogle Scholar
  56. 56.
    Zimmerman-Phillips S, Robbins AM, Osberger MJ. Assessing cochlear implant benefit in very young children. Ann Otol Rhinol Laryngol Suppl. 2000;109(12):42–3.Google Scholar
  57. 57.
    American Academy of Pediatrics Committee on Genetics. Health supervision for children with Down syndrome. Pediatrics. 2001;107:442–49.CrossRefGoogle Scholar
  58. 58.
    Roizen N, Patterson D. Down’s syndrome. Lancet. 2003;361(9365):1281–89.PubMedCrossRefGoogle Scholar
  59. 59.
    Mitchell RB, Call E, Kelly J. Ear nose and throat disorders in children with Down syndrome. Laryngoscope. 2003;113(2):259–63.PubMedCrossRefGoogle Scholar
  60. 60.
    Maurizi M, Ottaviani F, Paludetti G, Lungarotti S. Audiological findings in Down’s children. Int J Pediatr Otorhinolaryngol. 1985;9(3):227–32.PubMedCrossRefGoogle Scholar
  61. 61.
    Shott SR. Down syndrome: common otolaryngologic manifestations. Am J Med Genet C Semin Med Genet. 2006;142C(3):131–40.PubMedCrossRefGoogle Scholar
  62. 62.
    Balkany TJ, Mischke RE, Downs MP, Jafek BW. Ossicular abnormalities in Down’s syndrome. Otolaryngol Head Neck Surg. 1979;87(3):372–84.PubMedGoogle Scholar
  63. 63.
    Bilgin H, Kasemsuwan L, Schachern PA, Paparella MM, Le CT. Temporal bone studies of Down’s syndrome. Arch Otolaryngol Head Neck Surg. 1996;122(3):271–75.PubMedCrossRefGoogle Scholar
  64. 64.
    Blaser S, Propst EJ, Martin D, Feigenbaum A, James AL, Shannon P, Papsin BC. Inner ear dysplasia is common in children with Down syndrome (trisomy 21). Laryngoscope. 2006;116 12:2113–19.PubMedCrossRefGoogle Scholar
  65. 65.
    Shibahara Y, Sando I. Congenital anomalies of the Eustachian tube in Down syndrome. Histopathologic case report. Ann Otol Rhinol Laryngol. 1989;98 (7) Pt 1:543–47.PubMedGoogle Scholar
  66. 66.
    Centers for Disease Control and Prevention. Down syndrome prevalence at birth – United States, 1983-1990. MMWR. 1994;43(33):617–22.Google Scholar
  67. 67.
    Shin M, Besser LM, Kucik JE, Lu C, Siffel C, Correa A; Congenital Anomaly Multistate Prevalence and Survival Collaborative. Prevalence of Down syndrome among children and adolescents in 10 regions of the United States. Pediatrics. 2009;124(6):1565–71.PubMedCrossRefGoogle Scholar
  68. 68.
    American Speech-Language-Hearing Association. 2004. Guidelines for the audiologic assessment of children from birth to 5 years of age [Guidelines]. URL: http://www.asha.org/policy.
  69. 69.
    McDermott AL, Williams J, Kuo MJ, Reid AP, Proops DW. The role of bone anchored hearing aids in children with Down syndrome. Int J Pediatr Otorhinolaryngol. 2008;72(6):751–57.PubMedCrossRefGoogle Scholar
  70. 70.
    Hans PS, England R, Prowse S, Young E, Sheehan PZ. UK and Ireland experience of cochlear implants in children with Down syndrome. Int J Pediatr Otorhinolaryngol. 2010;74(3):260–64.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media B.V. 2011

Authors and Affiliations

  1. 1.Department of Speech Pathology and AudiologyWestern Michigan UniversityKalamazooUSA

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