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The child with a dual sensory loss (deafblind)


Introduction

Deafblindness or dual sensory loss (DSL) is a combined vision and hearing disability; affected children have complete or incomplete loss of hearing and/or vision. It can be congenital or acquired. Congenital deafblindness is defined as blind and deaf from birth or very early in life, before the development of speech (pre-lingual deafblindness). Acquired deafblindness arises after the development of language (post-lingual deafblindness). A Danish study found an overall prevalence of 1:19,000 for congenital deafblindness and 1:15,000 (including post-lingual deafblindness) among children <18 years of age. In the United States, the National Center on Deaf-Blindness (NCDB) performs a National Deaf-Blind Child Count annually. The 2018 count identified 7637 youngsters (3–18 years) and 647 infants (0–2 years); approximately 5% were totally blind and 20% had a profound hearing loss. Only about 1% had a profound hearing loss and were totally blind. Since 2014, these distributions have been rather stable, with an annual fluctuation of 1%–3%.

Etiology

Changes in health care have resulted in changes in the etiologies of congenital deafblindness. Rubella has diminished significantly due to immunization programs, and perinatal causes such as prematurity and perinatal asphyxia have increased with improvements in the survival of extremely premature and sick babies owing to better treatment options. Currently, the most common causes of congenital deafblindness are prematurity, CHARGE syndrome, Down syndrome, prenatal infection with cytomegalovirus (CMV), and meningitis. The most important cause of acquired deafblindness is Usher syndrome. Infections can cause early as well as late vision and/or hearing loss dependent on type and time of occurrence. Apart from that, many other syndromes and genetic disorders are involved in vision and hearing loss ( Table 99.1 ).

Table 99.1
Causes for deafblindness categorized in alphabetical order as: early (congenital or infantile) vision and hearing loss, early vision loss with subsequent hearing loss, early hearing loss with subsequent vision loss, and late vision and hearing loss
Early vision and hearing loss Early vision – subsequent hearing loss

  • Asphyxia

  • CHARGE association

  • Chromosomal

    • Chromosome 5p- (cri du chat)

    • Ring choromosome 18

    • Trisomy 4p, 13, 21 (Down syndrome)

  • Congenital infections

    • Cytomegalovirus (CMV)

    • Toxoplasmosis

    • Syphilis

    • Herpes simplex

    • Rubella

    • Meningitis

  • Premature birth

  • Other syndromes: Aicardi, Dandy–Walker, fetal alcohol, Smith–Lemni–Opitz, Waardenburg

  • Alström syndrome

  • Infections

  • Leber congenital amaurosis

  • Norrie disease

Early hearing – subsequent vision loss Late vision and hearing loss

  • CHARGE

  • Infections

  • Möbius syndrome

  • Prematurity

  • Stickler syndrome

  • Treacher Collins

  • Trisomy 21 (Down syndrome)

  • Usher type I, II syndromes

  • Chemical-induced

  • Infections

  • Intracranial pathology

  • Trisomy 4p

  • Trauma with severe head injury

  • Usher type II and III

  • Other syndromes: Alport, Apert, Bardet–Biedl, Batten, Cockayne, Cogan, Cornelia de Lange, Crigler–Najjar, Crouzon, Hunter, Hurler, Kearns, Sayre, Klippel–Feill, Kneist, Leigh, Marfan, Maroteaux–Lamy, Marshall, neurofibromatosis type 1 and 2, Pierre Robin, Pfeiffer, Prader–Willi, Refsum, Sturge–Weber, Turner, Zellweger

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