Hearing impairment and language delay in infants: Diagnostics and genetics

Abstract

This overview study provides information on important phoniatric and audiological aspects of early childhood hearing and language development with the aim of presenting diagnostic and therapeutic approaches. The article first addresses the universal newborn hearing screening that has been implemented in Germany for all infants since January 2009. The process of newborn hearing screening from the maternity ward to confirmation diagnostics is presented in accordance with a decision by the Federal Joint Committee (G-BA). The second topic is pediatric audiology diagnostics. Following confirmation of a permanent early childhood hearing disorder, the search for the cause plays an important role. Hereditary hearing disorders and intrauterine cytomegalovirus (CMV) infection, probably the most common cause of an acquired hearing disorder, are discussed and compared with the most common temporary hearing disorder, otitis media with effusion, which in some cases is severe enough to be relevant for hearing and language development and therefore requires treatment. The third topic covered in this article is speech and language development in the first 3 years of life, which is known today to be crucial for later language development and learning to read and write. There is a short overview and introduction to modern terminology, followed by the abnormalities and diagnostics of early speech and language development. Only some aspects of early hearing and language development are addressed here. Important areas such as the indication for a cochlear implant in the first year of life or because of unilateral deafness are not included due to their complexity.

Keywords: genetic hearing disorders; newborn hearing screening; organization of newborn hearing screening in Germany; pediatric audiology diagnosis; speech and language delay.

Table 1. Risk factors for early childhood hearing impairment according to recommendations of the Joint Committee on Infant Hearing [7]

Table 2. Hearing screening centers in Germany (states listed in alphabetical order), date 08/2013

Table 3. Risk factors for the development of otitis media with effusion

Table 4. Indications for prompt surgical middle ear correction of otitis media with effusion (Rosenfeld [89], Guidelines)

Table 5. Possible consequences of grommet insertion, modified from Vlastarakos and Rosenfeld [87], [89]

Table 6. Linguistic levels

Table 7. Language development in the first 3 years of life

Figure 1. Documentation of hearing screening from the examination booklet

(Federal Joint Committee (G-BA), legal entity under public law, Wegelystraße 8, 10623 Berlin)

Figure 2. Documentation sheet for the hearing screening center in Nordrhein

The documentation sheet is attached to the yellow examination booklet and includes the documentation and screening ID and contact addresses for parents with questions

Figure 3. Baby during TEOAE screening

Figure 4. AABR measurement in a newborn

Figure 5. Causes of hearing impairment, modified from Smith RJH [1]

Figure 6. CMV infection in pregnancy (http://www.cdc.gov/cmv/trends-stats.html)

Figure 7. Classification and prognosis of late talkers [90]

Figure 8. Classification of speech and language disorders, modified from Kauschke and Siegmüller [101]

Figure 9. Diagnostic sequence in patients presenting abnormal speech development at the age 21–24 months, according to Buschmann [99]