Phoniatrics I pp 857-961 | Cite as

Diagnosis and Differential Diagnosis of Disorders of Hearing Development

  • Ahmet Atas
  • Songul Aksoy
  • Antoinette am Zehnhoff-Dinnesen
  • Doris-Eva Bamiou
  • Sylva Bartel-Friedrich
  • Claire Benton
  • Hanno J. Bolz
  • Nicole G. Campbell
  • Frans Coninx
  • Martine de Smit
  • Jakub Dršata
  • Mona Hegazi
  • Armagan Incesulu
  • Kristin Kerkhofs
  • Arne Knief
  • Sabrina Kösling
  • Jill Massey
  • Peter Matulat
  • David R. Moore
  • Dirk Mürbe
  • Katrin Neumann
  • Haldun Oguz
  • Levent N. Ozluoglu
  • Waheeda Pagarkar
  • Ross Parfitt
  • Simona Poisson-Markova
  • Ewa Raglan
  • Charlotte Rogers
  • Mustafa Asim Safak
  • Pavel Seeman
  • Eva Seemanova
  • Tony Sirimanna
  • Piotr Swidzinski
  • Monika Tigges
  • Thomas Wiesner
Part of the European Manual of Medicine book series (EUROMANUAL)


Specific history-taking, drawing a family tree and thorough clinical examination are explained in detail. Standards and calibration in audiometry are presented. Pure-tone audiometry (PTA) and principles of masking, loudness tests and dead-region testing are outlined. Different test methods of behavioural measurements in children are provided.

Principles in assessing tinnitus in adults are complemented by paediatric tinnitus assessment.

Speech audiometry tests are presented. A hierarchical scheme for the diagnosis of auditory perception disorder is demonstrated.

As objective tests, acoustic immittance measurements and evoked response audiometry are explained in detail, the latter with focus on frequency specificity, achieved by specific signals or special response elaboration, the auditory steady-state responses. An overview is given concerning electro-audiometry.

Developmental balance assessment in children is explained from simple observation to detailed testing.

The assessment of communication mode, nasality and voice function, comprehension, lip reading, narrative skills, expressive levels, working memory and word finding are addressed, as well as auditory and visual perception, phonological awareness and literacy. An overview of occupational therapy examinations is provided.

Strategies for the aetiological assessment of hearing loss are defined. The imaging section focuses on congenital ear malformations, pediatric temporal bone tumours, tumour-like lesions and procedures in candidates for cochlear implantation. In genetic analysis, besides GJB2 testing, gene panel testing by massively parallel sequencing (MPS) has become the method of choice.

Audiological diagnostic challenges for the assessment of multiply handicapped children are discussed.

The last section focuses on parental reactions and coping strategies following the diagnosis of childhood hearing loss and on counselling and intervention programmes.


Audiological test procedures Subjective Age-specific Objective Tinnitus Balance Aetiological assessment 

Supplementary material

307062_1_En_16_MOESM1_ESM.mp4 (236.2 mb)
Video 16.1 Example of BOA responses (MP4 241831 kb)
307062_1_En_16_MOESM2_ESM.mp4 (198.5 mb)
Video 16.2 Recommended VRA procedure (MP4 203307 kb)


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Copyright information

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

Authors and Affiliations

  • Ahmet Atas
    • 1
    • 2
  • Songul Aksoy
    • 3
  • Antoinette am Zehnhoff-Dinnesen
    • 4
  • Doris-Eva Bamiou
    • 5
  • Sylva Bartel-Friedrich
    • 6
  • Claire Benton
    • 7
  • Hanno J. Bolz
    • 9
  • Nicole G. Campbell
    • 10
  • Frans Coninx
    • 11
  • Martine de Smit
    • 12
  • Jakub Dršata
    • 13
  • Mona Hegazi
    • 14
  • Armagan Incesulu
    • 15
  • Kristin Kerkhofs
    • 16
  • Arne Knief
    • 4
  • Sabrina Kösling
    • 17
  • Jill Massey
    • 18
  • Peter Matulat
    • 4
  • David R. Moore
    • 19
  • Dirk Mürbe
    • 20
  • Katrin Neumann
    • 21
  • Haldun Oguz
    • 22
  • Levent N. Ozluoglu
    • 23
  • Waheeda Pagarkar
    • 25
  • Ross Parfitt
    • 4
  • Simona Poisson-Markova
    • 26
  • Ewa Raglan
    • 24
  • Charlotte Rogers
    • 8
  • Mustafa Asim Safak
    • 27
  • Pavel Seeman
    • 26
  • Eva Seemanova
    • 26
  • Tony Sirimanna
    • 28
  • Piotr Swidzinski
    • 29
  • Monika Tigges
    • 30
  • Thomas Wiesner
    • 31
  1. 1.Department of Audiology, Faculty of Health ScienceIstanbul University CerrahpasaİstanbulTurkey
  2. 2.Department of ENT–Audiology and Speech Pathology, Faculty of Medicineİstanbul University CerrahpasaİstanbulTurkey
  3. 3.Department of AudiologyHacettepe UniversitySıhhiye-AnkaraTurkey
  4. 4.Clinic of Phoniatrics and PedaudiologyUniversity Hospital MünsterMünsterGermany
  5. 5.National Hospital for Neurology and NeurosurgeryLondonUK
  6. 6.Department of Otorhinolaryngology—Head and Neck SurgeryUniversity Hospital Martin Luther University Halle-WittenbergHalle (Saale)Germany
  7. 7.Nottingham Audiology Services, Ropewalk HouseNottinghamUK
  8. 8.Leicester School of Allied Health Sciences, De Montfort University, Hawthorn BuildingLeicesterUK
  9. 9.Senckenberg Centre for Human GeneticsFrankfurt am MainGermany
  10. 10.Auditory Implant ServiceUniversity of SouthamptonSouthamptonUK
  11. 11.Institut für Audiopädagogik, University of CologneSolingen-OhligsGermany
  12. 12.Artevelde University CollegeGhentBelgium
  13. 13.Department of Otorhinolaryngology and Head and Neck SurgeryUniversity Hospital Hradec KraloveHradec KraloveCzech Republic
  14. 14.ENT DepartmentAin Shams UniversityCairoEgypt
  15. 15.Faculty of Medicine, Department of Otolaryngology-Head and Neck SurgeryEskisehir Osmangazi UniversityEskişehirTurkey
  16. 16.Rehabilitationcentre De PoolsterBrusselsBelgium
  17. 17.Department of Diagnostic RadiologyMartin-Luther University Halle-WittenbergHalle (Saale)Germany
  18. 18.Evelina London Children’s Hospital, Guys and St Thomas’ NHS Foundation TrustLondonUK
  19. 19.Communication Sciences Research CenterCincinnati Children’s HospitalCincinnatiUSA
  20. 20.Department of Audiology and PhoniatricsCharité—University Medicine BerlinBerlinGermany
  21. 21.Department of Phoniatrics and Pediatric Audiology, ENT ClinicSt. Elisabeth Hospital, University of BochumBochumGermany
  22. 22.FonomerAnkaraTurkey
  23. 23.Department of OtolaryngologyBaskent UniversityEtimesgut/AnkaraTurkey
  24. 24.Department of Audiology and Audiovestibular MedicineGreat Ormond Street Hospital for Children NHS Foundation TrustLondonUK
  25. 25.Royal National Throat Nose & Ear Hospital, Nuffield Hearing and Speech CentreLondonUK
  26. 26.Department of Child Neurology2nd Medical School of Charles University PraguePragueCzech Republic
  27. 27.Department of OtolaryngologyNear East UniversityLefkosaTurkey
  28. 28.Audiological Medicine and Cochlear Implant DepartmentGreat Ormond Street HospitalLondonUK
  29. 29.Department of Phoniatrics and AudiologyPoznan University of Medical SciencesPoznanPoland
  30. 30.Städt. Klinikum Karlsruhe GmbH, ENT Clinic, Phoniatrics and PedaudiologyKarlsruheGermany
  31. 31.Department of Phoniatrics and Pediatric Audiology, Werner Otto Institut gGmbHHamburgGermany

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