Genetic hearing impairment: the primary cause of sensorineural hearing defects in the child.

Genetically-transferred hearing impairments account for more than 50% of ca ses of pediatric sensorineural hearing defects. Multiple clinical aspects a re involved in genetic hearing impairment, including the involvement of oth er organs, genetic inheritance, and the degree and age at onset of hearing loss. Diagnosis relies on family history on the systematic investigation of the symptomatology including an associated syndrome, and audiometry testin g in parents and siblings. Analysis of the connexin 26 gene is also indicat ed, as it is frequently involved in this disorder. Further genetic analysis in affected families will aid in detecting other as yet unidentified genes responsible for hearing impairment. (C) 2001 Editions scientifiques et med icales Elsevier SAS.