LYON _ The child deaf and after? To answer this question, the Dre Catherine Durandpediatrician and coordinator of the perinatal network of the two Savoy (RP2S), the Dre Catherine BlanchetOrl at the Montpellier University Hospital and the Dr Yannick LeroseyENT at the CH of Evreux and the Rouen University Hospital, detailed the tracking course, diagnosis and then care of deaf children, from maternity during a session of Congress of the French Pediatric Society (CSP 2025) .
Neonatal deafness screening has been generalized in France since 2014. “The problem is that its implementation has been entrusted to regional health agencies (ARS), without really national coordination”, specifies the Dre Catherine DurandReferent deafness screening with the Regional Union for the Prevention of Children’s Handicaps (URPHE), and vice-president of the French Federation of actors in neonatal auditory screening (FFADAN). There is also no deaf babies register in France.
She points out that this screening benefits from good societal acceptance. “There is almost no refusal of screening,” she says. The route begins from maternity, where a first test (T1) is systematically offered to parents. If it is not successful, a second test (T2) is carried out. And if it is again not satisfactory, a delayed test called T3 can be carried out via multiple actors (maternity/liberal/ENT). This delayed test is very often standardized.
At the end of the tests, we send 8 to 15 children per thousand in ENT and only one to two are diagnosed with deaf
“At this stage, if the test is always problematic, the child is sent to an ENT specialist, sometimes in an expert center, sometimes in liberal,” said Dr. Durand. Thus, the screening phase is carried out with automated tests and the diagnostic phase is reserved for specialists. In French maternities, T1 and T2 tests are mostly carried out with a device that measures either acoustic otoetmissions (Oea), or the potentials evoked Automated auditory (PEAA), which make it possible to determine if the auditory nerve works. “The latter are longer to do, but less often non-conclusive,” she explains.
Note that the HAS has just published new recommendations on screening for neonatal deafness which go in the direction of practices described by the speaker.
Decrease the use of ENT
“The test conditions are important, insists the ENT. You have to do the test in the presence of the parents and the baby is calm. The further we are, the better it works. The ideal is to achieve it at more than 48 hours of life ”. She advises to refer to the tool created by its network, baptized “Roulette des babés”, which indicates that doing at every hour of life of the child.
Hearing thresholds can change, in one direction or the other and it is necessary to make a regular evaluation
For T2 tests and the deferred T3 test, it recommends using the PEAA tests as much as possible, in order to preserve the specialized ENT resource, which are rare.
“The delayed outpatient test can be carried out at two or three weeks of life. It is a good timing for tests, which allows a catch-up of tests not done in maternity and decreases the use of specialists ENT, ”she underlines.
In 2021, the national perinatal survey showed that 100 % of maternities are scoring. According to the France Public Health Review for 2016, refusals were rare, less than one per thousand. After the screening phase, 1.4 % of babies remained suspected of bilateral disorder. In Auvergne Rhône-Alpes (aura) in the years 2022 to 2024, 1.51 % are suspected of unique or bilateral disorders. Entrance to the diagnostic phase concerned 62 % of children addressed in France in 2016, but 95 % over the past few years.
“At the end of the tests, we send 8 to 15 children per thousand in ENT and only one to two are diagnosed deaf,” she said.
Currently, screening and monitoring is only done in all regions for bilateral disorders.
“We believe that screening should be extended to unilateral damage for all babies. This is not the priority, but yet a unilateral disorder can be bilateralized, ”she notes.
“A proactive preventive attitude should be set up for the functional ear,” she insists. Hearing on both sides is important for neurode development, language, or even fatigability in noise. ” Nevertheless, this multiplies by 3 to 4 the number of babies to follow at the end of maternity, which can be a brake.
Be vigilant to choose from words
It warns on certain risk groups, such as premature babies. “It is the group most at risk of deafness, but their routes are complex and it is the group where there are the most lost sight of along the way,” she regrets. Babies born in an unconventional context, for example at home, can also be more difficult to detect. “For the babies of deaf parents, screening at birth is not necessarily the right time,” she adds.
The approach does not start from a parental doubt. Nothing can be seen. You must therefore be vigilant at the choice of words, their weight above the cradle.
In any case, you must have in mind that screening is not compulsory. “It must be explained in the parents’ language and respect a refusal, draw it and offer if it is better, direct access to the delayed test”. The psychological impact must also be taken into account: “The approach does not start from a parental doubt. Nothing can be seen. You must therefore be vigilant at the choice of words, their weight above the cradle. Non -conclusive test and diagnostic announcement should not be mixed. You have to explain, support, reassure parents without trivializing and knowing the courses and the actors, in order to be able to orient, ”recommends Dre Durand.
Important etiological diagnosis
The Dre Catherine BlanchetENT in the Montpellier University Hospital, has detailed the issues for the specialist. “It is first of all a question of confirming or invalidating the reality of deafness by checking in particular whether it is permanent or transient. It can indeed be due to an effusion, which spontaneously heals in two thirds of the cases. Then you have to characterize deafness: side, degree, etc. We must get hearing thresholds for each ear, ”she says.
Different tests must be used: otoscopy, potential evoked hearing (PEA), ASSR, PEA in bone conduction, Multifrequential Impedancemetry, OEA/DPOEA/Microphonic.
“No isolated test makes it possible to answer for sure,” she says.
“The repetition of tests is sometimes necessary. The initial diagnosis is only a first step. Hearing thresholds can change, in one direction or the other and it is necessary to make a regular evaluation, ”she says.
In addition, the etiological diagnosis is almost as important, as this influences management.
“Deafness can be due to an environmental factor (20-30 % of cases), such as a cmv maternity-fetal infection, or a genetic factor (70 to 80 % of cases), with isolated deafness non-syndromic or associated with syndrome) or an association of factors”.
She also highlights the importance of the psychological impact on parents.
The rehabilitation of congenital deafness is an emergency, because the function creates the organ
“The multiplicity of the teams, the repetition of the tests and the succession of ads can be very anxiety -provoking for the parents, who must manage uncertainty. When the diagnosis is made, there may be reactions of shock, amazement, denial. Parents must mourn the dream child. There is a risk of disruption of the parent-child relationship, hypervigilance or negative parental projections. Support is fundamental and requires the co-construction of a care project with families, ”she explains.
A race against the clock
The Dr Yannick LeroseyENT in the CH of Evreux and the CHU de Rouen, president of the French Federation of actors of the neonatal auditory screening (FFADAN), said this support.
“Early intervention is necessary, with a linguistic project established in cooperation between parents and professionals. Several stakeholders are necessary: ENT, hearing aidoprosthetist
psychologist, etc. “, He describes.
He insists on the need for information and a quick decision, especially if the project is an oralist.
“The rehabilitation of congenital deafness is an emergency, because the function creates the organ. These are the sounds that the ear will succeed in transmitting at the central level which will allow the development of hearing and language centers, ”he recalls.
The critical period for neuroplasticity is less than two years, which implies acting quickly. Thus, a hearing aid assessment and an apparatus must be set up, with an hearing career experienced in pediatrics.
“It can be implemented from the age of three months, with parents’ education and support. Monitoring with close-up checks, settings and frequent imprint to renew the tips in order to follow the growth of the child are essential, ”he notes.
The question that then arises is that of the efficiency of the equipment, or the need for a cochlear implant.
“The implant is indicated in severe or deep deafness, with an insufficient efficiency of hearing prostheses and without major malformation of the cochlea or the auditory nerve. The establishment must be carried out if possible before 1 year, ”he says.
Furthermore, “the implant or the prosthesis are not everything, these children will take years of speech therapy to have a development of language”, he completes.
The implant assessment is positive: excluding pathology associated, 80 % of children will have a level of language identical to their normal-enters peers, according to data from the High Authority for Health (HAS). But this requires several years of work. “The work carried out during the session must be repeated at home, so the active participation of the parents is essential,” adds Dr. Lerosey.
He concludes by insisting on “the race against the watch to win” in screening and taking care, in order to ensure the best chances for these deaf children.
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