Phoniatrics I pp 751-836 | Cite as

Basics of Disorders of Hearing Development

  • Antoinette am Zehnhoff-DinnesenEmail author
  • Wendy Albuquerque
  • Hanno J. Bolz
  • Steffi Johanna Brockmeier
  • Thorsten Langer
  • Radha Narayan
  • Ross Parfitt
  • Simona Poisson-Markova
  • Ewa Raglan
  • Sabrina Regele
  • Rainer Schönweiler
  • Pavel Seeman
  • Eva Seemanova
  • Amélie Elisabeth Tillmanns
  • Oliver Zolk
Part of the European Manual of Medicine book series (EUROMANUAL)


After the definition of hearing loss, audiological grading scales defining the presence and severity of hearing loss are presented. An overview is provided of epidemiological estimates of childhood hearing loss and issues involved in their calculation. After specification of the stages of hearing development, the different types of behavioural responses to sound that develop in normal children are described. An overview of the normal stages of speech and language development in normal-hearing children is followed by the impact of different kinds and degrees of hearing loss on speech and language acquisition of hearing-impaired children.

Risk factors for permanent childhood hearing impairment are listed, and likely additional disabilities are referred to. Infections with neurotropic viruses presenting complications and immunisation strategies are highlighted. A further aetiological key aspect concerns ototoxic side effects of anticancer drugs, anti-infective agents, loop diuretics and ototopical medications, which are presented in context with audiological diagnostics, monitoring and post-therapeutic follow-up, therapeutic options and preventive strategies. A third core area is related to syndromic and non-syndromic forms of hereditary hearing loss. More than 60 hearing loss syndromes are specified with their affected genes and chromosomal regions. Clinical manifestations with possible impact on hearing, ear-specific anomalies, the type of hearing impairment and cognitive function are described.


Definition Impact Audiological grading scales Epidemiology Development stages Aetiology 


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Copyright information

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

Authors and Affiliations

  • Antoinette am Zehnhoff-Dinnesen
    • 1
    Email author
  • Wendy Albuquerque
    • 2
  • Hanno J. Bolz
    • 3
  • Steffi Johanna Brockmeier
    • 4
  • Thorsten Langer
    • 5
  • Radha Narayan
    • 6
  • Ross Parfitt
    • 1
  • Simona Poisson-Markova
    • 7
  • Ewa Raglan
    • 8
  • Sabrina Regele
    • 1
  • Rainer Schönweiler
    • 9
  • Pavel Seeman
    • 7
  • Eva Seemanova
    • 7
  • Amélie Elisabeth Tillmanns
    • 1
  • Oliver Zolk
    • 10
  1. 1.Clinic of Phoniatrics and PedaudiologyUniversity Hospital MünsterMünsterGermany
  2. 2.Department of ENT and Audiovestibular Medicine, St Georges Healthcare NHS TrustLondonUK
  3. 3.Senckenberg Centre for Human GeneticsFrankfurt am MainGermany
  4. 4.ENT Clinic, Audiology, Phoniatrics, Neuro-OtologyKantonsspital Aarau AGAarauSwitzerland
  5. 5.Department of Pediatric Oncology and HematologyUniversity Hospital Schleswig-Holstein, Campus LübeckLübeckGermany
  6. 6.The Whittington HospitalLondonUK
  7. 7.Department of Child Neurology2nd Medical School of Charles University PraguePragueCzech Republic
  8. 8.Department of Audiology and Audiovestibular MedicineGreat Ormond Street Hospital for Children NHS Foundation TrustLondonUK
  9. 9.Department of Phoniatrics and Pediatric AudiologyUniversity Clinic of Schleswig-Holstein, Campus LübeckLübeckGermany
  10. 10.Institute of Pharmacology of Natural Products and Clinical Pharmacology, University Hospital UlmUlmGermany

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