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Diagnosis and Differential Diagnosis of Disorders of Hearing Development

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Phoniatrics I

Abstract

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.

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Example of BOA responses (MP4 241831 kb)

Video 16.2

Recommended VRA procedure (MP4 203307 kb)

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Atas, A. et al. (2020). Diagnosis and Differential Diagnosis of Disorders of Hearing Development. In: am Zehnhoff-Dinnesen, A., Wiskirska-Woznica, B., Neumann, K., Nawka, T. (eds) Phoniatrics I. European Manual of Medicine. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-46780-0_16

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