A sensory impairment that is most often present from birth

Most often present from birth, permanent neonatal hearing loss leads to disruptions in communication and language development, as well as cognitive development. Hearing loss in children is the most common sensory impairment, with an estimated prevalence of about 1 in 1,000 births.

Early and appropriate intervention can significantly reduce the impact of hearing loss on communication and language development, two key factors in a child’s academic success.
Given the positive impact of neonatal hearing screening, Santé publique France’s objectives are to evaluate the rollout of the national program for screening for permanent neonatal hearing loss, to propose ways to improve the implementation of this screening in France, and to inform the general public.

Newborn Screening

Thanks to technological advances over the past 20 years, hearing loss can be detected within the first few days of life. At the same time, increasingly effective and miniaturized hearing aids (hearing aids, implants, etc.) have been developed. In France, a universal neonatal hearing screening program has been in place since April 2012. Its goal is to lower the age at which children receive care in order to promote language and communication development in deaf children; this is done without prejudging the family’s choice regarding the approach selected (audio-phonatory, i.e., through the use of a hearing aid, or visual-gestural, through the acquisition of sign language). After informing the parents (or those with parental authority) and obtaining their consent, the program proceeds in two phases detailed in a decree published in November 2014:

• The screening phase, which is based on tests conducted during the hospital stay (in the maternity ward or the hospital department to which the child was transferred) and, in some cases, during a follow-up visit after discharge from the hospital.

• The diagnostic phase, which consists of a comprehensive hearing evaluation conducted in a specialized clinic.

An often unknown cause

Regarding the etiology of congenital hearing loss, a study¹ conducted by Fortnum et al. in 2002 on 17,160 English children showed that approximately 50% of cases are of genetic origin or acquired during a pre-, peri-, or postnatal infection, or through the administration of ototoxic substances during pregnancy. The remaining cases of congenital hearing loss are believed to have an etiology that remains unknown to this day, although medical research is identifying new genes associated with hearing loss, which, in the long term, should help reduce the proportion of cases with an undetermined cause.

Variable consequences

The medical and economic repercussions of hearing loss are well known. Several experts consulted by the HAS in 2007 noted that a delayed diagnosis of hearing loss consistently exacerbates communication difficulties, regardless of other factors involved in the development of communication and language acquisition.