Study: Genetic diagnosis helps to treat childhood hearing loss

Understanding the many different genetic causes of childhood-onset hearing loss suggests that genomic testing could aid in treatment planning, including optimal treatment timing. Even if a child with hearing loss did not receive a diagnosis from genetic testing a few years ago, scientists in this field believe that re-testing is worthwhile. Hearing loss, usually stemming from sensory problems in the inner ear, affects about 1 in 400 newborns in the United States. About half of these children have a genetic cause for their hearing loss. More than 120 genes and thousands of different genetic variants can lead to childhood hearing loss. Virtually all children with genetic hearing loss have a mutation or mutations in only one gene. A recent study, reported Jan. 12 in JAMA Otolaryngology-Head & Neck Surgery, examined a group of more than 400 youngsters with bilateral sensorineural hearing loss affecting both ears. The researchers from UW Medicine and Seattle Children’s Hospital sought to identify the genetic causes of hearing loss in this group and how each genetic cause relates to a specific type of hearing loss. They also evaluated whether the genetic cause of hearing loss was related to the success of treatment with a cochlear implant, a surgically placed electronic device that enables a person to sense sound through impulses transmitted to the brain. The findings show that genetic testing is a valuable tool in determining prognosis for a child’s hearing loss and in predicting how useful a cochlear implant could be for that child’s understanding of speech.