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Auditory Neuropathy Treatment & Management

  • Author: Wayne T Shaia, MD; Chief Editor: Arlen D Meyers, MD, MBA  more...
 
Updated: Jan 11, 2016
 

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 spectrum disorder, cochlear implants lead to improvements in hearing skills similar to those associated with cochlear implants in children with sensorineural hearing loss.[6]

A study by Liu et al found that children with AN 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.[7]

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.[8]

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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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Contributor Information and Disclosures
Author

Wayne T Shaia, MD Director, The Balance and Ear Center

Disclosure: Nothing to disclose.

Coauthor(s)

Dennis I Bojrab, MD CEO and Director of Research, Michigan Ear Institute; Chairman of Otolaryngology, William Beaumont Hospital; Director of Skull Base Surgery, Providence Hospital; Professor of Otolaryngology, Oakland University William Beaumont School of Medicine; Clinical Professor of Otolaryngology and Neurosurgery, Wayne State University School of Medicine

Dennis I Bojrab, MD is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American Medical Association, American Otological Society, North American Skull Base Society

Disclosure: Nothing to disclose.

Jason G May, MD Staff Physician, Department of Otolaryngology-Head and Neck Surgery, Harper Hospital/Wayne State University School of Medicine

Jason G May, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Otolaryngology-Head and Neck Surgery

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Gerard J Gianoli, MD Clinical Associate Professor, Departments of Otolaryngology-Head and Neck Surgery and Pediatrics, Tulane University School of Medicine; President, The Ear and Balance Institute; Board of Directors, Ponchartrain Surgery Center

Gerard J Gianoli, MD is a member of the following medical societies: American Otological Society, Society of University Otolaryngologists-Head and Neck Surgeons, Triological Society, American Neurotology Society, American Academy of Otolaryngology-Head and Neck Surgery, American College of Surgeons

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Vesticon<br/>Received none from Vesticon, Inc. for board membership.

Chief Editor

Arlen D Meyers, MD, MBA Professor of Otolaryngology, Dentistry, and Engineering, University of Colorado School of Medicine

Arlen D Meyers, MD, MBA is a member of the following medical societies: American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngology-Head and Neck Surgery, American Head and Neck Society

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Cerescan;RxRevu;SymbiaAllergySolutions<br/>Received income in an amount equal to or greater than $250 from: Symbia<br/>Received from Allergy Solutions, Inc for board membership; Received honoraria from RxRevu for chief medical editor; Received salary from Medvoy for founder and president; Received consulting fee from Corvectra for senior medical advisor; Received ownership interest from Cerescan for consulting; Received consulting fee from Essiahealth for advisor; Received consulting fee from Carespan for advisor; Received consulting fee from Covidien for consulting.

Additional Contributors

Michael E Hoffer, MD Director, Spatial Orientation Center, Department of Otolaryngology, Naval Medical Center of San Diego

Michael E Hoffer, MD is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery

Disclosure: Received royalty from American biloogical group for other.

References
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