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Meanwhile, professionals in the hearing field differ in their opinions about the potential benefits of hearing aids, cochlear implants, and other technologies for people with auditory neuropathy. Researchers are still seeking effective treatments for people with auditory neuropathy. Examples of such disorders are Charcot-Marie-Tooth syndrome and Friedreich’s ataxia. Some people with auditory neuropathy have neurological disorders that also cause problems outside of the hearing system. Thus, inheritance of mutated genes is also a risk factor for auditory neuropathy. Adults may also develop auditory neuropathy along with age-related hearing loss.Īuditory neuropathy runs in some families, and in some cases, scientists have identified genes with mutations that compromise the ear’s ability to transmit sound information to the brain. In addition, some drugs used to treat pregnant women or newborns may damage the baby’s inner hair cells, causing auditory neuropathy. These problems include inadequate oxygen supply during or prior to birth, premature birth, jaundice, low birth weight, and dietary thiamine deficiency. Some children diagnosed with auditory neuropathy experienced particular health problems before or during birth or as newborns. There are several ways that children may acquire auditory neuropathy. Sounds may fade in and out or seem out of sync for these individuals. For example, a person with auditory neuropathy may be able to hear sounds, but would still have difficulty recognizing spoken words. People with auditory neuropathy have greater impairment in speech perception than hearing health experts would predict based upon their degree of hearing loss on a hearing test. They always have poor speech-perception abilities, meaning that they have trouble understanding speech clearly. When their hearing sensitivity is tested, people with auditory neuropathy may have normal hearing or hearing loss ranging from mild to severe. The number of people affected by auditory neuropathy is not known, but current information suggests that auditory neuropathies play a substantial role in hearing impairments and deafness. It can affect people of all ages, from infancy through adulthood. Stronger evidence is needed to support cost-effective clinical policy and practice in this area.Auditory neuropathy is a hearing disorder in which the inner ear successfully detects sound, but has a problem with sending sound from the ear to the brain. Assessment of study quality, however, suggested substantial methodological concerns, particularly in relation to issues of bias and confounding, limiting the robustness of any conclusions around effectiveness.Ĭurrently available evidence is compatible with favourable outcomes from CI in children with ANSD. Outcomes following CI generally appeared similar in children with ANSD and SNHL. Speech recognition ability was found to be equivalent in CI users (who previously performed poorly with hearing aids) and hearing-aid users. Most children with ANSD achieved open-set speech recognition with their CI. This review aims to summarize and synthesize current evidence of the effectiveness of CI in improving speech recognition in children with ANSD.Ī total of 27 studies from an initial selection of 237.Īll selected studies were observational in design, including case studies, cohort studies, and comparisons between children with ANSD and SNHL. However, consensus has yet to be reached on its effectiveness for hearing loss caused by auditory neuropathy spectrum disorder (ANSD). Cochlear implantation (CI) is a standard treatment for severe-profound sensorineural hearing loss (SNHL).
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