Analysis of specific risk factors of neurodevelopmental disorder in hearing-impaired infants under ten months of age: “EnTNDre” an opening research stemming from a transdisciplinary partnership

• Published in: International journal of pediatric otorhinolaryngology Vol. 166; p. 111453
• Main Authors: Akrich, Salomé, Parlato de Oliveira, Erika, Favrot-Meunier, Claire, Rebichon, Caroline, de Clerck, Aurélie, Ettori, Sophie, Rouillon, Isabelle, Prang, Isabelle, Loundon, Natalie
• Format: Journal Article
• Language: English
• Published: Ireland Elsevier B.V 01.03.2023
• Subjects: Child; Children; Cytomegalovirus Infections - complications; Deafness - diagnosis; Early screening; Hearing; Hearing loss; Hearing Loss, Bilateral - complications; Hearing Loss, Sensorineural - genetics; Humans; Infant; Male; Neurodevelopmental disorder; Neurodevelopmental Disorders; Risk Factors; Early screening; Children; Neurodevelopmental disorder; Risk factors; Hearing loss
• ISSN: 0165-5876; 1872-8464
• DOI: 10.1016/j.ijporl.2023.111453

Approximately 20% of children born with severe to profound hearing loss (HL) have an associated disorder that poses a neurodevelopmental risk [1]. The objective of this study is to identify the criteria and profiles of deaf infants at risk of neurodevelopmental disorders (NDD) to provide early intervention. Twenty-two infants aged three to ten months with bilateral congenital deafness were included. Each child attended a consultation with a psychiatrist specializing in the development of hearing-impaired infants as part of their ENT follow-up. The quality of their early development was analyzed using the Olliac grid and well-known postural and sensorimotor criteria. The children were then classified into three groups: normal examination (Group 1), evident NDD (Group 2), and intermediate examination (Group 3). Early medical history, the etiology of deafness, cerebral imaging, and vestibular test results were collected and compared in the different groups. The average age of the children at the time of observation was seven months (3–10 months). All had sensorineural HL, with identified causes in 13 out of 22 cases: five cases of connexin 26 gene mutation, three cases of CHARGE syndrome, two CMV infections, one Usher syndrome, one GATA3 mutation, and one LHPL5 mutation. The average score on the Olliac grid was nine (0–15), and abnormal postural and sensorimotor behaviors were found in 15 cases out of 22 (68%). 27% of the children were classified in Group 1, 45% in Group 2, and 27% in Group 3. Children with non-isolated HL, abnormal brain MRI (8/22), malformations outside the auditory system (10/22), vestibular impairments (9/22), and/or CMV infections (2/22) were distributed as follows: 2/6 in Group 1, 9/10 in Group 2, and 3/6 in Group 3. 8/22 children had suffered perinatal complications (0/6 in Group 1, 6/10 in Group 2, and 2/5 in Group 3). Only one child had a first-degree relative with NDD. He belonged to Group 2. To our knowledge, this paper is the first to describe the development of infants with congenital deafness. It is based on an observation time that had been included in the procedure of multidisciplinary evaluations prior to cochlear implantation (CI), thanks to the partnership between a psychiatric center for deaf children and an ENT-pediatric implantology service. This consultation was aimed at assessing the quality of neurodevelopment and identifying NDD without a specific referral, with good acceptability for families. Using the Olliac grid and postural and sensorimotor criteria developed to be assessed in the routine care consultation, we identified evident early signs of NDD in 45% of infants. This group (Group 2) requires early, targeted, developmental support. Some children in an intermediate zone (Group 3) require further observation and support. The very early identification of NDD seems to be relevant in the care of hearing-impaired infants. The use of the Olliac grid and developmental scales seems relevant to identifying infants at risk for NDD.