eMedicine Specialties > Otolaryngology and Facial Plastic Surgery > Audiology

Auditory Neuropathy: Treatment & Medication

Author: Wayne T Shaia, MD, Consulting Staff, Department of Otology/Neurotology, Medical College of Virginia
Coauthor(s): Dennis I Bojrab, MD, Chairman of Otolaryngology, William Beaumont Hospital; Director of Skull Base Surgery, Providence Hospital; Professor, Department of Otolaryngology-Head and Neck Surgery, Department of Neurosurgery, Wayne State University; Jason G May, MD, Staff Physician, Department of Otolaryngology Head and Neck Surgery, Harper Hospital/Wayne State University School of Medicine
Contributor Information and Disclosures

Updated: Aug 27, 2009

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.

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. Most recently, if cochlear implantation fails, the option of brainstem implantation has been reported in one child. With the continued expansion of the indications of cochlear implantation, demonstration of the pathophysiology of AN/AD becomes more crucial in helping to determine which children are indeed good candidates for the increasingly popular surgical treatment of hearing loss.

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.

More on Auditory Neuropathy

Overview: Auditory Neuropathy
Differential Diagnoses & Workup: Auditory Neuropathy
Treatment & Medication: Auditory Neuropathy
Follow-up: Auditory Neuropathy
Multimedia: Auditory Neuropathy
References
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References

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  2. Buchman CA,, Roush PA,, Teagle HF et al. Auditory neuropathy characteristics in children with cochlear nerve deficiency. Ear Hear. 2006;Aug;27(4):399-408. [Medline].

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  4. Colletti V, Fiorino FG, Carner M, et al. Auditory brainstem implant as a salvage treatment after unsuccessful cochlear implantation. Otol Neurotol. Jul 2004;25(4):485-96; discussion 496. [Medline].

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Further Reading

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Keywords

auditory neuropathy, auditory dyssynchrony, AD, AN, AN/AD, AD/AN, central processing disorder, pediatric hearing loss, hearing loss, cochlear implants, otoacoustic emissions, Friedrich ataxia, Stevens-Johnson syndrome, Ehlers-Danlos syndrome, Charcot-Marie-Tooth syndrome, hyperbilirubinemia, anoxia, hypoxia

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Contributor Information and Disclosures

Author

Wayne T Shaia, MD, Consulting Staff, Department of Otology/Neurotology, Medical College of Virginia
Disclosure: Nothing to disclose.

Coauthor(s)

Dennis I Bojrab, MD, Chairman of Otolaryngology, William Beaumont Hospital; Director of Skull Base Surgery, Providence Hospital; Professor, Department of Otolaryngology-Head and Neck Surgery, Department of Neurosurgery, Wayne State University
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, and 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 and American Academy of Otolaryngology-Head and Neck Surgery
Disclosure: Nothing to disclose.

Medical Editor

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: American biloogical group Royalty Other

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Gerard J Gianoli, MD, Clinical Associate Professor, Department of Otolaryngology-Head and Neck Surgery, Tulane University School of Medicine; Vice President, The Ear and Balance Institute; Chief Executive Officer, Ponchartrain Surgery Center
Gerard J Gianoli, MD is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American College of Surgeons, American Neurotology Society, American Otological Society, Society of University Otolaryngologists-Head and Neck Surgeons, and Triological Society
Disclosure: Nothing to disclose.

CME Editor

Christopher L Slack, MD, Otolaryngology-Facial Plastic Surgery, Private Practice, Associated Coastal ENT; Medical Director, Treasure Coast Sleep Disorders
Christopher L Slack, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngology-Head and Neck Surgery, and American Medical Association
Disclosure: Nothing to disclose.

Chief Editor

Arlen D Meyers, MD, MBA, Professor, Department of Otolaryngology-Head and Neck Surgery, 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, and American Head and Neck Society
Disclosure: Covidien Corp Consulting fee Consulting; US Tobacco Corporation unstricted gift unknown; Axis Three Corporation Ownership interest Consulting; Omni Biosciences Ownership interest Consulting; Sentegra Ownership interest Board membership; Syndicom Ownership interest Consulting; Oxlo  Consulting; Medvoy Ownership interest Management position

 
 
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