Phoniatrics I pp 963-981 | Cite as

Prevention of Disorders of Hearing Development

  • Antoinette am Zehnhoff-DinnesenEmail author
  • Hanno J. Bolz
  • Gwen Carr
  • Manfred Gross✝
  • Ross Parfitt
  • Simona Poisson-Markova
  • Debbie Rix
  • Pavel Seeman
  • Eva Seemanova
  • Monika Tigges
Part of the European Manual of Medicine book series (EUROMANUAL)


Universal newborn hearing screening (UNHS) has turned out to be the most effective way to identify congenital paediatric sensorineural hearing loss. Screening principles and methods, possible screening failures, management of control intervals, screenings for late-onset hearing loss and how to communicate the results of UNHS are core areas of the first section.

A brief overview is provided of some concepts and existing formal programmes that aim to support parents in enhancing parent-child communication skills.

Audiograms of family members can provide an indication of the type of hearing disorder and possible genetic causes. Affected relatives may profit from earlier diagnosis and intervention.

Genetic counselling is crucial in the diagnostic approach of hereditary hearing loss. Results of DNA testing should be explained to the patient, his parents and eventually other relatives by a clinical geneticist.

In conclusion, this chapter presents information on advice concerning hearing conservation, especially in the case of large endolymphatic duct and sac, and how to prevent noise- or music-induced hearing loss. The necessity to avoid noise in combination with ototoxic drugs is a further issue. Some general aspects are added.


Hearing screening Enhancing parental communication skills Family audiometry Genetic counselling Hearing conservation 


  1. American Academy of Otolaryngology, Head and Neck Surgery (2017) Noise-induced hearing loss in children. Available via Accessed 17 June 2017
  2. ASHA (2016). Accessed 27 June 2016
  3. Barreira-Nielsen C, Fitzpatrick E, Hashem S et al (2016) Progressive hearing loss in early childhood. Ear Hear 37(5):e311–e321PubMedCrossRefGoogle Scholar
  4. Bartel-Friedrich S, Amaya B, Rasinski C et al (2008a) Der erweiterte Ductus und Saccus endolymphaticus. Teil 1: Analyse bildgebender Befunde. (Large endolymphatic duct and sac syndrome (LEDS): part I: analysis of imaging findings). HNO 56(2):219–224PubMedCrossRefGoogle Scholar
  5. Bartel-Friedrich S, Fuchs M, Amaya B et al (2008b) Der erweiterte Ductus und Saccus endolymphaticus. Teil 2: Klinische Manifestationen. (Large endolymphatic duct and sac syndrome: part 2: clinical manifestations). HNO 56(2):225–230PubMedCrossRefGoogle Scholar
  6. Baxendale J, Hesketh A (2003) Comparison of the effectiveness of the Hanen Parent Programme and traditional clinic therapy. Int J Lang Commun Dis 38(4):397–415CrossRefGoogle Scholar
  7. Beazley S, Moore M (1995) Deaf children, their families and professionals. David Fulton Publishers, LondonGoogle Scholar
  8. Brown ED, Chau JK, Atashband S et al (2009) A systematic review of neonatal toxoplasmosis exposure and sensorineural hearing loss. Int J Pediatr Otorhinolaryngol 73(5):707–711. Epub 2009 Feb 11CrossRefPubMedGoogle Scholar
  9. Cabot RC, Genter CR, Lucke T (1979) Sound levels and spectra of rock music. J Audio Eng Soc 27:267–283Google Scholar
  10. Center for Hearing and Communication (2017) International Noise Awareness Day 23rd anniversary. Available via Accessed 17 June 2017
  11. Centers for Disease Control and Prevention (2016) Hearing protector device compendium. Available via Accessed 17 June 2017
  12. Choo D, Meinzen-Derr J (2010) Universal newborn hearing screening in 2010. Curr Opin Otolaryngol Head Neck Surg 18(5):399–404. Scholar
  13. Colgan S, Gold L, Wirth K et al (2012) The cost-effectiveness of universal newborn screening for bilateral permanent congenital hearing impairment: systematic review. Acad Pediatr 12(3):171–180. Scholar
  14. Cristobal R, Oghalai JS (2008) Hearing loss in children with very low birth weight: current review of epidemiology and pathophysiology. Arch Dis Child Fetal Neonatal Ed 93(6):F462–F468. Scholar
  15. Dangerous Decibels (2017) Noise-induced hearing loss can be prevented. Available via Accessed 17 June 2017
  16. De Barros Boishardy A, Lenoir FM, Kapella M et al (2005) Universal hearing screening: 10,835 newborns tested in maternity wards of the geographical Department of Eure, France. Ann Otolaryngol Chir Cervicofac 122(5):223–230PubMedCrossRefGoogle Scholar
  17. de Vries JJ, Vossen AC, Kroes AC et al (2011) Implementing neonatal screening for congenital cytomegalovirus: addressing the deafness of policy makers. Rev Med Virol 21(1):54–61. Epub 2011 Jan 18CrossRefPubMedGoogle Scholar
  18. DesJardin JL (2006) Family empowerment: Supporting language development in young children who are deaf or hard of hearing. Volta Rev 106(3):275–298Google Scholar
  19. Downs MP, Yoshinaga-Itano C (1999) The efficacy of early identification and intervention for children with hearing impairment. Pediatr Clin N Am 46(1):79–87CrossRefGoogle Scholar
  20. Easterbrooks SR, O’Rourke CM, Todd NW (2000) Child and family factors associated with deaf children’s success in auditory-verbal therapy. Am J Otol 21(3):341–344PubMedCrossRefGoogle Scholar
  21. European Board of Medical Genetics (2010) Core competences for genetic counsellors. Available via Accessed 8 Dec 2016
  22. European Union (2003) Directive 2003/19/EC. Available via Accessed 17 June 2017
  23. Fligor B (2012) Clinical verification of custom-fitted musicians’ earplugs. Available via Audiology Online. Accessed 12 Apr 2017
  24. Gandhi RS, Fernandez-Alvarez JR, Rabe H (2010) Management of congenital cytomegalovirus infection: an evidence-based approach. Acta Paediatr 99(4):509–515. Epub 2009 Dec 24CrossRefPubMedGoogle Scholar
  25. Gilles A, Van de Heyning P (2014) Effectiveness of a preventive campaign for noise-induced hearing damage in adolescents. Int J Pediatr Otorhinolaryngol 278(4):604–609CrossRefGoogle Scholar
  26. Gilles A, De Ridder D, Van Hal G et al (2012) Prevalence of leisure noise-induced tinnitus and the attitude toward noise in university students. Otol Neurotol 33(6):899–906PubMedGoogle Scholar
  27. Granell J, Gavilanes J, Herrero J et al (2008) Is universal newborn hearing screening more efficient with auditory evoked potentials compared to otoacoustic emissions? Acta Otorrinolaringol Esp 59(4):170–175PubMedCrossRefGoogle Scholar
  28. Gross M (2005) Universelles Hörscreening bei Neugeborenen—Empfehlungen zu Organisation und Durchführung des universellen Neugeborenen-Screenings auf angeborene Hörstörungen in Deutschland. (Universal hearing screening in newborns—recommendations for organizing and conducting universal hearing screening for congenital hearing loss in Germany). Laryngorhinootologie 84(11):801–808PubMedCrossRefGoogle Scholar
  29. Grosse SD, Ross DS (2006) Cost savings from universal newborn hearing screening. Pediatrics 117(4):1101–1112CrossRefGoogle Scholar
  30. Harrison RV (2008) Noise-induced hearing loss in children: a ‘less than silent’ environmental danger. Paediatr Child Health 13(5):377–382PubMedPubMedCentralCrossRefGoogle Scholar
  31. Harrison RV (2012) The prevention of noise induced hearing loss in children. Int J Pediatr 2012:473541. Published online 2012 Dec 13. Scholar
  32. Holzinger D, Wieshaupt A, Fellinger P et al (2016) Prevalence of 2.2 per mille of significant hearing loss at school age suggests rescreening after NHS. Int J Pediatr Otorhinolaryngol 87:121–125PubMedCrossRefGoogle Scholar
  33. Janjua F, Woll B, Kyle J (2002) Effects of parental style of interaction on language development in very young severe and profound deaf children. Int J Pediatr Otorhinoalaryngol 64(3):193–205CrossRefGoogle Scholar
  34. Joint Committee on Infant Hearing (1995) 1994 position statement. Pediatrics 95(1):152–156Google Scholar
  35. Joint Committee on Infant Hearing (2000) Year 2000 position statement: principles and guidelines for early hearing detection and intervention programs. Pediatrics 106(4):798–817CrossRefGoogle Scholar
  36. Kaye CI (2006) Introduction to the newborn screening fact sheets. Pediatrics 118(3):1304–1312PubMedCrossRefGoogle Scholar
  37. Keith SE, Michaud DS, Chiu V (2008) Evaluating the maximum playback sound levels from portable digital audio players. J Acoust Soc Am 123(6):4227–4237PubMedCrossRefGoogle Scholar
  38. Kochhar A, Hildebrand MS, Smith RJ (2007) Clinical aspects of hereditary hearing loss. Genet Med 9(7):393–408PubMedCrossRefPubMedCentralGoogle Scholar
  39. Konigsmark BW, Gorlin RJ (eds) (1976) Genetic and metabolic deafness. WB Saunders, PhiladelphiaGoogle Scholar
  40. le Clercq CM, van Ingen G, Ruytjens L et al (2016) Music-induced hearing loss in children, adolescents, and young adults: a systematic review and meta-analysis. Otol Neurotol 37(9):1208–1216PubMedCrossRefGoogle Scholar
  41. Li H, Steyger PS (2009) Synergistic ototoxicity due to noise exposure and aminoglycoside antibiotics. Noise Health 11(42):26–32PubMedPubMedCentralCrossRefGoogle Scholar
  42. Liu X, Xu L (1994) Nonsyndromic hearing loss: an analysis of audiograms. Ann Otol Rhinol Laryngol 103(6):428–433PubMedCrossRefGoogle Scholar
  43. Louw C, Swanepoel W, Eikelboom RH et al (2017) Smartphone-based hearing screening at primary health care clinics. Ear Hear 38(2):e93–e100PubMedCrossRefGoogle Scholar
  44. Low WK, Pang KY, Ho LY et al (2005) Universal newborn hearing screening in Singapore: the need, implementation and challenges. Ann Acad Med Singap 34(4):301–306PubMedGoogle Scholar
  45. Martini A, Marchisio P, Bubbico L et al (2013) Permanent childhood hearing impairment: universal newborn hearing screening, PCHI management. Minerva Pediatr 65(2):231–250PubMedGoogle Scholar
  46. Moeller MP (2000) Early intervention and language development in children who are deaf and hard of hearing. Pediatrics 106(3):E43PubMedPubMedCentralCrossRefGoogle Scholar
  47. Moeller MP, Carr G, Seaver L et al (2013) Best practices in family-centered early intervention for children who are deaf or hard of hearing: an international consensus statement. J Deaf Stud Deaf Educ 18(4):429–445. Scholar
  48. Morton CC, Nance WE (2006) Newborn hearing screening—a silent revolution. N Engl J Med 354(20):2151–2164PubMedCrossRefGoogle Scholar
  49. Münzel T, Gori T, Babisch W et al (2014) Cardiovascular effects of environmental noise exposure. Eur Heart J 35(13):829–836PubMedPubMedCentralCrossRefGoogle Scholar
  50. National Institute on Deafness and Other Communication Disorders (2017) Enlarged vestibular aqueducts and childhood hearing loss. Available via Accessed 17 June 2017
  51. Neumann K, Nawka T, Wiesner T et al (2009) Autorengruppe im Auftrag der DGPP: Qualitätssicherung eines universellen Neugeborenen-Hörscreenings. Empfehlungen der Deutschen Gesellschaft für Phoniatrie und Pädaudiologie. (Quality assurance of a universal newborn hearing screening. Recommendations of the German Society of Phoniatrics and Pediatric Audiology). HNO 57(1):17–20PubMedCrossRefGoogle Scholar
  52. Noordman BJ, van Beeck Calkoen E, Witte B et al (2015) Prognostic factors for sudden drops in hearing level after minor head injury in patients with an enlarged vestibular aqueduct: a meta-analysis. Otol Neurotol 36(1):4–11PubMedGoogle Scholar
  53. Olusanya BO, Wirz SL, Luxon LM (2008) Hospital-based universal newborn hearing screening for early detection of permanent congenital hearing loss in Lagos, Nigeria. Int J Pediatr Otorhinolaryngol 72(7):991–1001PubMedCrossRefGoogle Scholar
  54. Oyler AL (2007) Large vestibular aqueduct (LVA) disorders. ASHA. Available via Accessed 17 June 2017
  55. Portnuff CDF (2016) Reducing the risk of music-induced hearing loss from overuse of portable listening devices: understanding the problems and establishing strategies for improving awareness in adolescents. Adolesc Health Med Ther 7:27–35PubMedPubMedCentralCrossRefGoogle Scholar
  56. Portnuff CDF, Fligor BJ, Arehart KH (2011) Teenage use of portable listening devices: a hazard to hearing? J Am Acad Audiol 22(10):663–677PubMedCrossRefGoogle Scholar
  57. Prpić I, Mahulja-Stamenković V, Bilić I et al (2007) Hearing loss assessed by universal newborn hearing screening-the new approach. Int J Pediatr Otorhinolaryngol 71(11):1757–1761PubMedCrossRefGoogle Scholar
  58. Ross DS, Holstrum WJ, Gaffney M et al (2008) Hearing screening and diagnostic evaluation of children with unilateral and mild bilateral hearing loss. Trends Amplif 12(1):27–34. Scholar
  59. Sataloff RT (1998) Rock concert audience noise exposure: a preliminary study. J Occup Hear Loss 1:97–99Google Scholar
  60. Schmitz J, Pillion JP, LeClerq SC et al (2010) Prevalence of hearing loss and ear morbidity among adolescents and young adults in rural southern Nepal. Int J Audiol 49(5):388–394PubMedCrossRefGoogle Scholar
  61. Sekhar DL, Rhoades JA, Longenecker AL et al (2011) Improving detection of adolescent hearing loss. Arch Pediatr Adolesc Med 165(12):1094–1100PubMedCrossRefGoogle Scholar
  62. Shearer AE, Hildebrand MS, Smith RJH (2017) Hereditary hearing loss and deafness overview. Gene Reviews [Internet]. Available via Accessed 28 Nov 2017
  63. Skarżyński H, Piotrowska A (2012) Screening for pre-school and school-age hearing problems: European Consensus Statement. Int J Pediatr Otorhinolaryngol 76(1):120–121PubMedCrossRefGoogle Scholar
  64. Smith RJ, Bale JF Jr (2005) Sensorineural hearing loss in children. Lancet 365(9462):879–890PubMedCrossRefGoogle Scholar
  65. Tharpe AM, Sladen DP (2008) Causation of permanent unilateral and mild bilateral hearing loss in children. Trends Amplif 12(1):17–25PubMedPubMedCentralCrossRefGoogle Scholar
  66. The Hearing Foundation of Canada (2013) Sound sense. Available via Accessed 17 June 2017
  67. Toriello HV, Reardon W, Gorlin RJ (2004) Hereditary hearing loss and its syndromes. Oxford Monographs on Medical Genetes no. 50. Oxford University Press, OxfordGoogle Scholar
  68. Tranebaerg L (2008) Genetics of congenital hearing impairment: a clinical approach. Int J Audiol 47(9):535–545. Scholar
  69. US Preventive Services Task Force (2008) Universal screening for hearing loss in newborns: US Preventive Services Task Force recommendation statement. Pediatrics 122(1):143–148CrossRefGoogle Scholar
  70. Vogel I, Verschuure H, van der Ploeg CP et al (2009) Adolescents and MP3 players: too many risks, too few precautions. Pediatrics 123(6):e953–e958. Scholar
  71. Vos B, Lagasse R, Levêque A (2013) The organisation of universal newborn hearing screening in the Wallonia-Brussels Federation. B-ENT Suppl 21:9–15Google Scholar
  72. Weichbold V, Nekahm-Heis D, Welzl-Mueller K (2006) Universal newborn hearing screening and postnatal hearing loss. Pediatrics 117(4):e631–e636PubMedCrossRefGoogle Scholar
  73. White KR (2004) Early hearing detection and intervention programs: opportunities for genetic services. Am J Med Genet A 130A(1):29–36PubMedCrossRefGoogle Scholar
  74. Wilkinson AR, Jiang ZD (2006) Brainstem auditory evoked response in neonatal neurology. Semin Fetal Neonatal Med 11(6):444–451. Epub 2006 Oct 2PubMedCrossRefGoogle Scholar
  75. Wolff R, Hommerich J, Riemsma R et al (2010) Hearing screening in newborns: systematic review of accuracy, effectiveness, and effects of interventions after screening. Arch Dis Child 95(2):130–135PubMedCrossRefGoogle Scholar
  76. Women’s and Children’s Health Network (2017) Parenting and child health. Child and youth health. Noise and hearing. Available via Accessed 17 June 2017
  77. World Health Organization (2009) Children and noise. Children’s health and the environment. Available via www.who.intr/ceh/capacity/noise. Accessed 17 June 2017
  78. Yee-Arellano HM, Leal-Garza F, Pauli-Muller K (2006) Universal newborn hearing screening in Mexico: results of the first 2 years. Int J Pediatr Otorhinolaryngol 70(11):1863–1870. (PubMed: 16914209)PubMedCrossRefGoogle Scholar
  79. Yoshinaga-Itano C (2000) Successful outcomes for deaf and hard of hearing children. Semin Hear 21:4CrossRefGoogle Scholar
  80. Yoshinaga-Itano C, Sedey AL, Coulter DK et al (1998) Language of early- and later-identified children with hearing loss. Pediatrics 102(5):1161–1171PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2020

Authors and Affiliations

  • Antoinette am Zehnhoff-Dinnesen
    • 1
    Email author
  • Hanno J. Bolz
    • 2
  • Gwen Carr
    • 3
  • Manfred Gross✝
    • 4
  • Ross Parfitt
    • 1
  • Simona Poisson-Markova
    • 5
  • Debbie Rix
    • 6
  • Pavel Seeman
    • 5
  • Eva Seemanova
    • 5
  • Monika Tigges
    • 7
  1. 1.Clinic of Phoniatrics and PedaudiologyUniversity Hospital MünsterMünsterGermany
  2. 2.Senckenberg Centre for Human GeneticsFrankfurt am MainGermany
  3. 3.UCL Ear Institute, University College LondonLondonUK
  4. 4.Department of Audiology and PhoniatricsCampus Virchow-KlinikumBerlinGermany
  5. 5.Department of Child Neurology2nd Medical School of Charles University PraguePragueCzech Republic
  6. 6.Wandsworth Hearing Support Service, Linden Lodge SchoolLondonUK
  7. 7.Städt. Klinikum Karlsruhe GmbH, ENT Clinic, Phoniatrics and PedaudiologyKarlsruheGermany

Personalised recommendations