Newborn hearing screening is currently being implemented in the United States and other countries, allowing early identification of and intervention for hearing loss in neonates. Also, genetic testing is clinically available for the GJB2 gene, which codes for the connexin 26 protein, and the results have begun to explain the cause of a significant number of cases of hearing loss. However, the current limited knowledge about the natural history of GJB2-related hearing loss in the postnatal period, particularly as it relates to age at diagnosis of the hearing loss, raises important clinical and ethical questions that need to be addressed regarding the evaluation of prelingual siblings of children identified with GJB2-related hearing loss.
Palmer CGS, Martinez A, Sininger Y, Shapiro N, Grody WW, Schimmenti LA. Prelingual Siblings of Children With GJB2 Hearing LossIssues to Consider. Arch Otolaryngol Head Neck Surg. 2005;131(11):1020-1022. doi:10.1001/archotol.131.11.1020