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Congenital deafness or hearing loss is hearing loss that is present at birth. This hearing loss can either be manifested at birth or develop later in life, usually during childhood. Relatively rare, congenital hearing loss occurs in approximately <\/span>one out of every 1,000 to 2,000 births<\/span><\/a>. Approximately 50 percent of <\/span>congenital hearing loss<\/span><\/a> cases are due to genetics, 25 percent are\u00a0due to acquired, non-hereditary causes at birth, and 25 percent of cases have unknown causes.<\/p>\n <\/span><\/p>\n Contents<\/p> \u00a0<\/p>\n Some cases of congenital hearing loss arise due to <\/span>non-hereditary reasons<\/span><\/a> that are present during pregnancy or at birth. These reasons can include:<\/span><\/p>\n <\/span><\/li>\n<\/ul>\n \u00a0<\/p>\n The majority of congenital deafness or hearing loss cases are due to genetic or hereditary reasons. This hearing loss can be present at birth or it may develop later. Both parents may have healthy hearing yet carry recessive genes that cause issues or genetic syndromes\u00a0in which hearing loss is a symptom. Some genetic syndromes that manifest in congenital deafness or hearing loss include:<\/span><\/p>\n <\/span>\n<\/li>\n<\/ul>\n \u00a0<\/p>\n Treatment for congenital deafness or hearing loss is often determined after weighing several factors including the age of the child, the child\u2019s developmental level, the degrees of hearing loss, and cost of treatment. It is recommended that <\/span>treatment for congenital hearing loss<\/span><\/a> start before the age of six months. At this age, children are beginning to develop communication skills. In order for the child to develop at a\u00a0similar level to normal hearing children, aids to hearing are crucial.<\/span><\/p>\n Many causes of congenital hearing loss or deafness are due to incorrectly formed inner or outer ears during fetal development. For many of these malformations that are causing congenital hearing loss, surgery may be an option to correct some of these problems. An otolaryngologist (ear, nose, and throat doctor) can often perform corrective surgery to restore some or all hearing to a child suffering from these hearing issues.<\/p>\n <\/span><\/p>\n \u00a0<\/p>\n For some types of congenital hearing loss, hearing aids can be used to improve hearing. Hearing aids can be fitted at a very early age, and the younger the better is recommended to ensure the proper development of speech perception, speech production, and spoken language skills. Behind the ear (BTE) style hearing aids are recommended because there is less of a swallowing risk compared to smaller styles like in-the-ear, and BTE hearing aids are easier to adjust and fit as the child grows. <\/span><\/p>\n Bilateral hearing aids are recommended to facilitate binaural hearing, which allows the child to localize sound and better understand speech in noisy environments. Audiologist appointments will be much more frequent than an adults, usually every three months, in order to adjust and fit hearing aids to the child\u2019s rapidly growing ear canal.<\/p>\n <\/span><\/p>\n \u00a0<\/p>\n For children whose hearing loss is so severe that hearing aids do not help, cochlear implants may help improve their hearing. Cochlear implants are used when problems with the ear canal, middle ear, or inner ear, prevent that normal sound transmission pathway from working entirely. Cochlear implants bypass the normal pathway completely and deliver sound directly to the cochlea, which then transmits sound information to the auditory nerve.<\/span><\/p>\n Cochlear implants can help severely hearing impaired or deaf children develop language skills, including language comprehension and expression. For children with a damaged or nonfunctional cochlea or auditory nerve, an auditory brainstem implant may help provide some level of hearing. Auditory brainstem implants bypass the inner ear and auditory nerve and instead directly stimulate the hearing pathways in the brain. <\/span><\/p>\n Whether a cochlear implant or auditory brainstem implant is used, It is recommended that children be implanted as early as possible to maximize auditory rehabilitation.<\/p>\n <\/span><\/p>\n <\/p>\n The information in this guide has been written using the following reliable sources:<\/p>\n <\/b><\/p>\n https:\/\/rarediseases.org\/rare-diseases\/usher-syndrome\/<\/span><\/a><\/p>\n https:\/\/www.californiaearinstitute.com\/ear-disorders-treacher-collins-syndrome-bay-area.php<\/span><\/a><\/p>\n https:\/\/medlineplus.gov\/genetics\/condition\/alport-syndrome\/<\/span><\/a><\/p>\n https:\/\/rarediseases.org\/rare-diseases\/crouzon-syndrome\/<\/span><\/a><\/p>\n https:\/\/medlineplus.gov\/genetics\/condition\/waardenburg-syndrome\/<\/span><\/a><\/p>\n http:\/\/www.raisingdeafkids.org\/hearingloss\/causes\/<\/span><\/a><\/p>\n https:\/\/www.ncbi.nlm.nih.gov\/pmc\/articles\/PMC5222593\/<\/span><\/a><\/p>\n https:\/\/www.ncbi.nlm.nih.gov\/pmc\/articles\/PMC5675031\/<\/span><\/a><\/p>\n https:\/\/www.dizziness-and-balance.com\/disorders\/hearing\/cong_hearing.html<\/span><\/a><\/p>\n https:\/\/www.asha.org\/public\/hearing\/congenital-hearing-loss\/<\/span><\/a><\/p>\nAcquired Causes of Congenital Deafness<\/b><\/span><\/h2>\n
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Genetic Causes of Congenital Deafness<\/b><\/span><\/h2>\n
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How is Congenital Deafness Treated?<\/b><\/span><\/h2>\n
Hearing Aid Help\u00a0<\/strong><\/span><\/h2>\n
Cochlear Implants for Congenital Deafness<\/strong><\/span><\/h2>\n