A variety of conditions can cause loss of hearing in a newborn infant. More than half of profound congenital hearing loss is believed to be genetic in origin. For every child with a profound hearing loss, there are an estimated 1 or 2 children with lesser but clinically significant bilateral or unilateral loss. Genetic deafness is usually caused either by the possession of a single gene (dominant deafness) or 2 genes (recessive deafness). Genetic causes of hearing loss can be categorized as syndromic (having symptoms in addition to hearing loss, such as blindness or heart defects) or nonsyndromic (having no additional symptoms). The majority of genetic cases of hearing loss are nonsyndromic, autosomal recessive. The major environmental (nongenetic) cause of hearing loss is congenital cytomegalovirus (CMV) infection, a condition that occurs in 1 in 150 live births. The risk for permanent disability is highest when a woman becomes infected with CMV during the first trimester of pregnancy. Infection control practices to prevent exposure to CMV must be followed by all women who are pregnant or trying to become pregnant.
Universal newborn hearing screening is becoming the standard of care in the United States. The US Preventive Services Task Force (USPSTF) reported in July 2008 that there is scientific evidence to recommend newborn hearing screening for all infants, updating their statement of 2001. Universal newborn hearing screening is the first step in the national Early Hearing Detection and Intervention (EHDI) program.
The Joint Committee on Infant Hearing Loss 2007: Principles of EHDI state:
All infants should have access to hearing screening using a physiologic measure before 1 month of age.
All infants who do not pass the initial hearing screen and the subsequent rescreening should have appropriate audiologic and medical evaluations, to confirm the presence of hearing loss, before 3 months of age.
All infants with confirmed permanent hearing loss should receive intervention services before 6 months of age. A simplified, single point of entry into an intervention system appropriate to children with hearing loss is optimal.
The EHDI system should be family-centered with infant and family rights and privacy guaranteed through informed choice, shared decision making, and parental consent. Families should have access to information about all intervention and treatment options and counseling with regard to hearing loss.
The child and family should have immediate access to high-quality technology, including hearing aids, cochlear implants, and other assistive devices when appropriate.
All infants and children should be monitored for hearing loss in the medical home. Continued assessment of communication development should be provided by appropriate providers to all children with or without risk indicators for hearing loss.
Appropriate interdisciplinary intervention programs for deaf and hard-of-hearing infants and their families should be provided by professionals who are knowledgeable about childhood hearing loss. Intervention programs should recognize and build on strengths, informed choices, traditions, and cultural beliefs of the families.
Information systems should be designed to interface with electronic health records and should be used to measure outcomes and report the effectiveness of EHDI services at the community, state, and federal levels.
The goal of newborn hearing screening is to identify hearing problems early so that infants will develop the best communication and language skills possible. Midwives and other obstetric providers should educate women in regard to CMV infection, its possible effects on the newborn, and infection control practices to reduce the risk of acquiring CMV during pregnancy. This information is particularly important for women who are planning to become pregnant.