Medscape is available in 5 Language Editions – Choose your Edition here.


Auditory Neuropathy Workup

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

Laboratory Studies

No hematologic workup is necessary to diagnose auditory neuropathy/auditory dyssynchrony (AN/AD). History and audiologic testing establish the diagnosis (see History and Other Tests).


Imaging Studies

See the list below:

  • Imaging studies are not necessary in the newborn period.
  • Once the diagnosis is made correctly, conservative treatment can be initiated. If the parents choose surgical intervention, high-resolution computed tomography scanning of the temporal bones should be performed. This test helps the otologist or neurootologist determine the possibilities of inner ear malformations that might contribute to the disorder. In addition, the inner ear can be visualized and preparations for cochlear implantation can be made.[3]
  • Typically, magnetic resonance imaging (MRI) has no role in AN/AD.

Other Tests

See the list below:

  • The most pertinent audiologic tests for auditory neuropathy/auditory dyssynchrony (AN/AD) are briefly summarized, as follows[4, 5] :
    • Pure tone audiogram testing: This is a graphic plot of a patient's thresholds of auditory sensitivity for pure tone (sine wave) stimuli. It does not test a patient's ability to process sound. This test shows only the patient's ability to hear sounds or tones.
    • Speech audiometry: These tests use spoken words and sentences rather than pure tones. Tests are designed to assess sensitivity (threshold) or understanding (intelligibility) of speech.
    • Acoustic reflex (AR) measures: This measures the contraction of the stapedius muscle in the middle ear. Deviation from the normal threshold on AR testing indicates potential abnormalities of the hearing nerve and auditory system.
    • Otoacoustic emissions (OAEs): These are measured by the presentation of a series of clicks to the ear through a probe inserted in the ear canal. This test measures the integrity of the outer hair cells of the cochlea and cochlear function. Cochlear microphonics (CMs) tests the function of the cochlea similarly.
    • Auditory brainstem responses (ABRs): This test uses scalp electrodes to measure electrical activity in response to sound clicks. Abnormal results of ABR testing indicate that the hearing nerve, as well as the brainstem nuclei, may not correctly process the sounds.
  • Criteria for the diagnosis of AN/AD are as follows:
    • With the advent of newborn hearing screens, children with auditory detriments are being identified and treated at increasingly earlier ages. All of the following must be present in newborns to diagnose AN/AD:
      • Absent or severely abnormal ABR test results at maximal stimulus (100 dBnHL)
      • Normal outer hair cell function as determined by OAEs or CMs
      • Absent or elevated stapedial reflex thresholds
    • Suspect AN/AD in older children or adults with the following audiologic findings:
      • Pure tone thresholds are abnormal. The entire range of abnormalities, from near-normal to profound, may be seen. A more severe loss is usually displayed in the lower frequency thresholds.
      • Poor speech discrimination scores are out of proportion with the level of loss suspected based on the pure tone average.
      • The audiogram findings may vary some, but the overall milieu usually remains unchanged.
Contributor Information and Disclosures

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

Disclosure: Nothing to disclose.


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.

  1. Berlin CI, Hood L, Rose K. On renaming auditory neuropathy as auditory dys-synchrony. Auditory Today. 2002. 13:15-17.

  2. Berlin CI, Hood LJ, Morlet T, Wilensky D, Li L, Mattingly KR, et al. Multi-site diagnosis and management of 260 patients with auditory neuropathy/dys-synchrony (auditory neuropathy spectrum disorder). Int J Audiol. 2010 Jan. 49(1):30-43. [Medline].

  3. Roche JP, Huang BY, Castillo M, Bassim MK, Adunka OF, Buchman CA. Imaging characteristics of children with auditory neuropathy spectrum disorder. Otol Neurotol. 2010 Jul. 31(5):780-8. [Medline].

  4. Mo L, Yan F, Liu H, Han D, Zhang L. Audiological results in a group of children with auditory neuropathy spectrum disorder. ORL J Otorhinolaryngol Relat Spec. 2010. 72(2):75-9. [Medline].

  5. Hood LJ. Auditory Neuropathy/Dys-Synchrony Disorder: Diagnosis and Management. Otolaryngol Clin North Am. 2015 Dec. 48 (6):1027-40. [Medline].

  6. Fernandes NF, Morettin M, Yamaguti EH, Costa OA, Bevilacqua MC. Performance of hearing skills in children with auditory neuropathy spectrum disorder using cochlear implant: a systematic review. Braz J Otorhinolaryngol. 2015 Jan-Feb. 81 (1):85-96. [Medline].

  7. Liu Y, Dong R, Li Y, et al. Effect of age at cochlear implantation on auditory and speech development of children with auditory neuropathy spectrum disorder. Auris Nasus Larynx. 2014 Dec. 41 (6):502-6. [Medline].

  8. Teagle HF, Roush PA, Woodard JS, Hatch DR, Zdanski CJ, Buss E, et al. Cochlear implantation in children with auditory neuropathy spectrum disorder. Ear Hear. 2010 Jun. 31(3):325-35. [Medline].

  9. 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].

  10. Buss E, Labadie RF, Brown CJ, et al. Outcome of cochlear implantation in pediatric auditory neuropathy. Otol Neurotol. 2002 May. 23(3):328-32. [Medline].

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

  12. Conlee JW, Shapiro SM. Morphological changes in the cochlear nucleus and nucleus of the trapezoid body in Gunn rat pups. Hear Res. 1991 Dec. 57(1):23-30. [Medline].

  13. Harrison RV. An animal model of auditory neuropathy. Ear Hear. 1998 Oct. 19(5):355-61. [Medline].

  14. Kraus N, Ozdamar O, Stein L, Reed N. Absent auditory brain stem response: peripheral hearing loss or brain stem dysfunction?. Laryngoscope. 1984 Mar. 94(3):400-6. [Medline].

  15. Madden C, Hilbert L, Rutter M, et al. Pediatric cochlear implantation in auditory neuropathy. Otol Neurotol. 2002 Mar. 23(2):163-8. [Medline].

  16. Madden C, Rutter M, Hilbert L, et al. Clinical and audiological features in auditory neuropathy. Arch Otolaryngol Head Neck Surg. 2002 Sep. 128(9):1026-30. [Medline].

  17. Merchan-Perez A, Liberman MC. Ultrastructural differences among afferent synapses on cochlear hair cells: correlations with spontaneous discharge rate. J Comp Neurol. 1996 Jul 22. 371(2):208-21. [Medline].

  18. Narne VK, Vanaja CS. Perception of envelope-enhanced speech in the presence of noise by individuals with auditory neuropathy. Ear Hear. 2009 Feb. 30(1):136-42. [Medline].

  19. Nikolopoulos TP. Auditory dyssynchrony or auditory neuropathy: understanding the pathophysiology and exploring methods of treatment. Int J Pediatr Otorhinolaryngol. 2014 Feb. 78(2):171-3. [Medline].

  20. Peterson A, Shallop J, Driscoll C, et al. Outcomes of cochlear implantation in children with auditory neuropathy. J Am Acad Audiol. 2003 May-Jun. 14(4):188-201. [Medline].

  21. [Guideline] Sanyelbhaa Talaat H, Kabel AH, Samy H, Elbadry M. Prevalence of auditory neuropathy (AN) among infants and young children with severe to profound hearing loss. Int J Pediatr Otorhinolaryngol. Jul 2009. 73(7):937-9. [Medline].

  22. Shaia WT, Shapiro SM, Spencer RF. The jaundiced gunn rat model of auditory neuropathy/dyssynchrony. Laryngoscope. 2005. 115(12):2167-73. [Medline].

  23. Sininger YS, Trautwein P. Electrical stimulation of the auditory nerve via cochlear implants in patients with auditory neuropathy. Ann Otol Rhinol Laryngol Suppl. 2002 May. 189:29-31. [Medline].

  24. Spencer RF, Shaia WT, Gleason AT, et al. Changes in calcium-binding protein expression in the auditory brainstem nuclei of the jaundiced Gunn rat. Hear Res. 2002 Sep. 171(1-2):129-141. [Medline].

  25. Sutton G, Gravel J, Hood L, et al. Assessment and Management of Auditory Neuropathy/Auditory Dys-synchrony. UK Newborn Hearing Screening. 2005. 1-13. [Full Text].

Anatomy of the external and middle ear.
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.