Congenital Hearing Loss

Doctor showing an elderly person a hearing aid

Congenital hearing loss refers to hearing loss that is present at birth. It can be any degree of hearing loss, from mild to profound. Congenital hearing loss can be unilateral or bilateral.

What causes congenital hearing loss?

The two types of causes of congenital hearing loss are genetic (hereditary) and nongenetic (nonhereditary).

Genetic hearing loss accounts for 75% of congenital hearing loss and more than 50% of all hearing loss.

Nongenetic hearing loss accounts for about 25% of congenital hearing loss. There are several causes of a nongenetic congenital hearing loss, including:

  • Premature birth or low birth weight
  • Mondini malformation- malformation of the Cochlea
  • Toxemia or anoxia during pregnancy
  • Maternal infections
  • Maternal diabetes
  • Enlarged vestibular aqueduct syndrome

How is congenital hearing loss diagnosed?

Every state in the United States has an established newborn hearing screening program in place. This ensures that every newborn is screened for hearing loss before they leave the hospital. The aim of the United States Early Hearing Detection and Intervention (EHDI) program is to diagnose a hearing loss before 3 months of age and begin treatment of a child with permanent hearing loss by 6 months of age.

There are two non-invasive objective tests used to screen a newborn's hearing: Otoacoustic Emission (OAE) and Auditory Brainstem Response (ABR).

How is it treated?

Treatment for a congenital hearing loss will depend on the type and degree of the hearing loss. In most cases, hearing aids or implantable hearing devices will be used to manage the child's permanent hearing loss.

Does congenital hearing loss cause lasting problems?

If a congenital hearing loss is left untreated, it will delay a child's speech and language development.

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