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Hearing loss and deafness in the pediatric population: causes, diagnosis, and rehabilitation

Paul Deltenre1, Lionel Van Maldergem

  • 1Auditory Neurophysiology Laboratory, Department of Neurology and Rehabilitation, Université Libre de Bruxelles, Brugmann Hospital, Brussels, Belgium.

Handbook of Clinical Neurology
|April 30, 2013
PubMed

Insights

Early identification of childhood hearing loss is crucial for development. Objective testing and prompt intervention, including genetic evaluation and multidisciplinary rehabilitation, significantly mitigate the impact of this common sensory impairment.

Area of Science:

  • Pediatrics
  • Audiology
  • Genetics

Background:

  • Childhood hearing loss affects nearly 0.2% of newborns and 0.35% of adolescents, impacting speech, language, and cognitive development.
  • Early detection is vital as hearing loss can be a silent handicap with long-term consequences.
  • Acquired causes like cytomegalovirus (CMV) infections and genetic factors contribute significantly to hearing impairment.

Purpose of the Study:

  • To emphasize the importance of early diagnosis for childhood hearing loss.
  • To highlight the role of objective physiological techniques in identifying hearing loss mechanisms.
  • To discuss the management of acquired and genetic causes, including the need for genetic testing guidelines and timely interventions.

Main Methods:

  • Utilizing objective physiological techniques such as evoked potentials, oto-acoustic emissions, and tympanometry.
  • Applying the cross-check principle for accurate diagnosis.
  • Reviewing acquired causes (CMV, aminoglycoside ototoxicity, meningitis) and genetic etiologies.

Main Results:

  • Objective testing provides crucial insights into the mechanism of hearing loss and neural code disruption.
  • Genetic factors account for over 60% of congenital hearing loss, with new causes continually being identified.
  • Syndromic hearing loss requires prompt neuropediatric attention for effective management.

Conclusions:

  • Early and accurate diagnosis of childhood hearing loss is essential for mitigating developmental interference.
  • A multidisciplinary approach involving rehabilitation, assistive devices (hearing aids, cochlear implants), and parental involvement is key to optimal outcomes.
  • Guidelines for genetic testing are necessary due to the heterogeneity of genetic hearing loss causes.

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