CLINICAL TOPICS

Permanent Childhood Hearing Loss

Overview

Incidence and Prevalence

Signs and Symptoms

Causes

Nongenetic Factors

Nongenetic factors of permanent hearing loss include

  • maternal infections (e.g., rubella [German measles], cytomegalovirus, or herpes simplex virus),
  • prematurity,
  • low birth weight,
  • birth injuries,
  • toxins, including drugs and alcohol consumed by the mother during pregnancy,
  • complications associated with the Rh factor in the blood/jaundice,
  • maternal diabetes,
  • toxemia during pregnancy,
  • lack of oxygen (anoxia),
  • inner ear malformations (e.g., Mondini's malformation, large vestibular aqueduct),
  • outer/middle ear malformations (Atresia, stenosis).

Genetic Factors

Hearing loss from genetic causes can be present at birth or develop later in life. Most genetic hearing loss can be described as autosomal recessive or autosomal dominant. Other, rarer types of genetic hearing loss include X-linked (related to the sex chromosome) or mitochondrial inheritance patterns.

Genetic syndromes have a group of signs and symptoms that together indicate a specific disease. Many genetic syndromes include hearing loss as one of the symptoms. In fact, 20% of babies with genetic hearing loss have a syndrome (Morton & Nance, 2006). Examples of genetic disorders that include hearing loss are

  • Down syndrome,
  • Usher syndrome,
  • Treacher Collins syndrome,
  • Crouzon syndrome,
  • Alport syndrome,
  • Sickle cell disease,
  • Tay-Sachs disease,
  • Waardenburg syndrome,
  • Pendred syndrome,
  • Goldenhar syndrome,
  • CHARGE syndrome.

Acquired Hearing Loss

Acquired hearing loss occurs after birth. Conditions that may cause permanent acquired hearing loss in children include

  • ear infections,
  • ototoxic medications,
  • meningitis,
  • measles,
  • encephalitis,
  • chicken pox,
  • mumps,
  • large vestibular aqueduct,
  • head injury,
  • noise exposure.

Roles and Responsibilities

Assessment

Treatment

Resources

References

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