Language and Speech Perception Outcomes in Hearing-Impaired Children with and without Connexin 26 Mutations

Abstract

This study addressed the question of whether a hearing loss caused by mutations in the connexin 26 gene had a significant effect on language and speech perception outcomes in children using cochlear implants or hearing aids. The families of children who had participated in two previous studies of language development were invited to participate in this genetic study. From the 52 children whose families agreed to participate, 15 children with connexin 26 mutations in both chromosomes were identified. After taking into account other factors which are known to affect language development and speech perception in children with impaired hearing, no significant differences were found between the 15 children where connexin 26 was known to be the cause of deafness and the other 37 children in the study.

Speech Perception Outcomes after Cochlear Implantation in Children with GJB2/DFNB1 associated Deafness

“…The results show that cochlear implantation is effective in the development of speech perception after cochlear implantation in GJB2-related deafness to a similar extent as in deafness due to unknown etiology. These findings are in agreement with findings obtained by other authors (4,13,(15)(16)(17)(18)(19)(20)(21)(22). A recent long term follow-up of cochlear implantation in children with GBJ2-related deafness in Japan showed similar developments in speech performance in comparison with hearing loss due to other etiologies (23).…”

Associação entre fenótipo, desempenho com próteses auditivas e genótipo da deficiência auditiva infantil em crianças com e sem alteração genética

“…Some authors studied the relationship among the use of auditory aids (performance and benefit), language development and speech perception with different manifestations of HI with genetic origin (4)(5)(6) . The results of the three mentioned studies converge to a similar finding, that the genetic mutations do not determine directly the benefit with sound amplification.…”

“…Some studies aim at clarifying the relationship between the use of hearing aids (performance and benefit), language development and speech perception and the different manifestations of hereditary hearing impairments (4)(5)(6) . Other authors (7)(8)(9)(10) studied the performance of subjects with cochlear implantation, according to their hearing impairments, observing that children with genetic alterations present more benefits with cochlear implantation.…”

Cochlear Implants in Children: A Review

“…Many children with severe-profound hearing loss, including those with cochlear implants, have 4 to 5 year delays in spoken language development by the time they enter secondary school (Blamey et al, 2001a;Dahl et al, 2003;Davis & Hind, 1999;Ramkalawan & Davis, 1992;Sarant et al, 2009). Generally, the greater the degree of hearing loss, the larger the language delay (Boothroyd et al, 1991).…”