Auditory Neuropathy Treatment & Management

Updated: May 15, 2018
  • Author: Wayne T Shaia, MD; Chief Editor: Arlen D Meyers, MD, MBA  more...
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Treatment

Medical Care

The treatment of patients with auditory neuropathy/auditory dyssynchrony (AN/AD) starts with the parents. Information should be made available to all parents of children with hearing loss. Once this is done and the condition is thoroughly understood, the proper supportive adjuvant therapies can begin. These include speech pathology, hearing aid placement, and use of other hearing devices. The use of hearing aids can begin with children at around age 3 months.

Children with AN/AD were once thought not to benefit from hearing aid amplification; however, recent studies demonstrate that 50% of children can benefit from placement of an amplification device. When children with AN/AD were tested with hearing aids, their speech discrimination scores improved and were more consistent with the degree of hearing loss expected via their pure tone audiometry scores. The use of hearing aids prior to cochlear implantation is currently recommended.

Once the child is aged approximately 6 months, behavior audiometry thresholds should be obtained. Because the presentation and thresholds of AN/AD are so varied, the determination of more accurate levels of hearing loss helps to dictate the future intervention necessary for each child.

Communicative devices, which are options for any child with mild-to-severe hearing loss, also pertain to children with AN/AD. The use of conventional hearing aids and frequency modulation (FM) systems can help a child develop necessary speech and language skills. If a child does not progress with hearing aid devices and shows limited speech discrimination abilities, cochlear implantation is the next viable option.

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Surgical Care

Cochlear implants were approved in 1984 by the US Food and Drug Administration for use in adults. Six years later, the approval expanded to children, and inclusion criteria expanded to include larger groups of individuals with hearing impairment. Today, approximately 6000 cochlear implants are placed annually for various causes of hearing loss. In 2001, the use of cochlear implantation was expanded to include children with AN.

To date, only several hundred cochlear implants have been surgically placed in children and adults with AN/AD. However, long-term results have been promising, with demonstrations that children with AN/AD and implants had equivalent hearing abilities to other children of similar age with implants.

A literature review by Fernandes et al indicated that in children with AN/AD spectrum disorder, cochlear implants lead to improvements in hearing skills similar to those associated with cochlear implants in children with sensorineural hearing loss. [9]

A study by Liu et al found that children with AN/AD spectrum disorder who received cochlear implants prior to age 24 months tended to show better development of auditory and speech skills than did children who received the implants at a later age. [10]

If cochlear implantation fails, another option may exist in AN/AD, with brainstem implantation having been reported. With the continued expansion of indications for cochlear implantation, demonstration of the pathophysiology of AN/AD has become more crucial in helping to determine which children are indeed good candidates for the increasingly popular surgical treatment of hearing loss. [11]

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Consultations

Children with hearing loss require special attention. A multidisciplinary approach had been adopted by most, which includes an otologist or neurootologist, speech pathologist, genetic counselor, audiologist, and, possibly, a pediatric neurologist and neonatologist.

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