Among risk factors of neurodevelopmental disorder is congenital hearing loss. However, congenital deafness is neither necessary nor sufficient for the occurrence of warning signs of neurodevelopmental disorder. The investigative team hypothesizes that the co-occurrence of these two clinical entities results from a common origin, within a syndromic diagnosis including other medical issues. These situations would therefore be very different from those of babies suffering from isolated sensorineural deafness.

This study aims to identify which factors are statistically correlated with the association of congenital deafness and early symptoms of neurodevelopmental disorder, thanks to a partnership between a regional center for psychiatry of deaf children (Therapeutic Childhood and Deafness Unit of St Maurice Hospitals, UTES) and a pediatric audiophonology department (ENT department of the Necker-Enfants Malades hospital).

This work is based on a cohort of 26 children under ten months of age diagnosed with severe to profound congenital sensorineural hearing loss. As part of their pre-implantation cochlear assessment, a consultation was filmed to precisely analyze the interactional and sensorimotor skills of these children (clinical observations, administration of a specific evaluation grid called Olliac Grid), after reviewing the films. The investigative team will then be able to grade the neurodevelopmental risk.

In order to clarify what factors are linked with a neurodevelopmental risk, medical data concerning the child's family context, birth, and audiological profile will also be collected, and put in perspective with the assessed neurodevelopmental risk gradation.

Congenital deafness remains among well-known risk factors for Neurodevelopmental Disorders, in particular Autism Spectrum Disorders (ASD). According to current data of the literature, the risk of ASD in children with hearing loss is twice as high as in the general population. Furthermore, while the main aim of French government strategy for autism 2018-2022 is to promote early detection and intervention, it is noted that deaf children suffer from a significant diagnostic delay with regard to autism spectrum disorders.

Today it is possible to detect clinical warning signs of a neurodevelopmental impairment since the first months of life. The investigative team believe that these warning signs present a risk for further severe disability such as ASD, Attention Deficit / Hyperactivity Disorder (ADHD), learning disorders and so on, that is to say every psychiatric diagnosis included in neurodevelopmental disorders as the DSM-V classifies them. This encourages clinicians to assess every new-born with high risk factors of neurodevelopmental disorder.

No research has been conducted on the screening of Autism Spectrum Disorders (and more broadly of Neurodevelopmental Disorders) in deaf infants and the correlations with their clinical profile (health conditions, psychomotor development and typology of deafness).

One of the difficulties lies in the fact that these children constitute a heterogeneous population. Studying deaf infants requires taking into account multiple parameters, such as etiology of the hearing loss, associated disorders, or early life history. Moreover, first months of a born-deaf child are a highly emotional and demanding period for parents. However, the investigative team hope that observing this population will help better understand what constitutes in newborns with congenital hearing loss, associated risk factors for autism.

Knowing that congenital deafness is neither necessary nor sufficient for the early occurrence of a neurodevelopmental warning signs, the investigative team hypothesizes that the co-occurrence of these two medical conditions results from a common origin, as a syndromic diagnosis including other medical issues. These situations would therefore be very different from those of babies suffering from isolated sensorineural deafness.

The investigative team aim to identify which factors are statistically correlated with the association of congenital deafness and early diagnosis of clinical signs of neurodevelopmental risk, thanks to a partnership between a regional center for psychiatry of deaf children (Therapeutic Childhood and Deafness Unit of St Maurice Hospitals, UTES) and an infant audiophonology department (ENT department of the Necker-Enfants Malades hospital).

This work is based on a cohort of 26 infants under ten months of age diagnosed with severe to profound congenital sensorineural hearing loss. The enrollment of the patients took place from May 2018 to April 2020, based on consultations for the management of deafness in the audiophonology department of the Necker Enfants Malades Hospital in Paris.

As part of the pre-implantation cochlear assessment carried out in the audiophonology department of the Necker Enfants Malades Hospital, each child in the cohort has attended a consultation to assess the risk of neurodevelopmental disorder. This pilot consultation was carried out by a child psychiatrist from the UTES together with a psychologist from the ENT department of Necker, and was filmed to precisely analyze the interactional skills of the children (clinical observations, administration of a specific evaluation grid called Olliac Grid (Olliac et al, 2017), gradation of the neurodevelopmental risk), after reviewing the films.

Anamnesis data concerning the family context (parental and sibling history) and birth (antenatal conditions, term, per and post-natal complications) will be collected, with particular attention to the elements already identified in the literature as associated with risk of Neurodevelopmental Disorders. In order to clarify what factors are linked with a neurodevelopmental risk, medical data concerning the child's family context, birth, and audiological profile will be collected, and put in perspective with the previously assessed neurodevelopmental risk gradation.

Inclusion Criteria:

• Children with congenital deafness
• Follow-ups in the ENT department of the Necker hospital
• Having benefited from a pilot consultation, filmed, jointly carried out by a child psychiatrist from Therapeutic Childhood and Deafness Unit of St Maurice Hospitals (UTES) and a psychologist from the Necker ENT department for the assessment of neurodevelopmental risk
• Pilot consultation carried out before the child is 10 months old
• Information of the holders of parental authority and collection of their non-opposition to the study

Exclusion Criteria:

• Children assessed after 10 months
• Opposition of legal representatives for the use of their child's medical data and of the film

Children with congenital deafness followed in the ENT department of the Necker hospital and having benefited before their 10 months, from a pilot consultation, filmed, jointly carried out by a child psychiatrist from the Therapeutic Childhood and Deafness Unit of St Maurice Hospitals Therapeutic Childhood and Deafness Unit of St Maurice Hospitals (UTES) and a psychologist from the ENT department of Necker for the neurodevelopmental risk assessment.