Brain, Vol. 119, No. 3, 741-753, 1996
© 1996 Guarantors of Brain
research-article |
Auditory neuropathy
1Department of Neurology, University of Califomia Irvine 2House Ear Institute Las Angeles 3Kresge Hearing Research Laboratory, Louisiana State University Medical Center New Orleans, USA 4Rotman Reseach Institute of Baycress Centre, University of Toronto Toronto, Canada
Correspondence to:
Correspondence to: Arnold Starr, Department Neurology, University of Califomia Irvine, Irvine, CA 92717. USA
Ten patients presented as children or young adults with hearning impairments that, by behavioural and physiological testing, were compatible with a disorder of the auditory portion of the VIII cranial nerve. Evidence of normal cochlear outer hair cell function was provided by preservation of otoacoustic emissions and cochlear microphonics in all of the patients. Auditory brainstem potentials showed evidence of abnormal auditory pathway function beginning with the VIII nerve: the potentials were absent in nine patients and severely distorted in one patient. Auditory brainstem reflexes (middle ear muscles: crossed suppression of otoacoustic emissions) were absent in all of the tested patients. Behavioural audiometric testing showed a mild to moderate elevation of pure tone threshold in nine patients. The extent of the hearing loss, if due to cochlear receptor damage, should not have resulted in the loss of auditory brainatem potentials. The shape of the pure tone loss varied, being predominantly low frequency in five patients, flat across all frequencies in three patients and predominantly high frequency in two patients. Speech intelligibility was tested in eight patients, and in six was affected out of proportion to what would have been expected if the pure tone loss were of cochlear origin. The patients were otherwise neurologically normal when the hearing impairment was first manifest. subsequently, eight of these patients developed evidence for a peripheral neuropathy. The neuropathy was hereditary in three and sporadic in five. We suggest that this type of hearing impairment is due to a disorder of auditory nerve function and may have, as one of its causes, a neuropathy of the auditory nerve, occurring either in isolation or as part of a generalized neuropathic process.
neural hearing loss; auditory neuropathy
Received June 21, 1995. Revised December 1, 1995. Accepted January 12, 1996.
CiteULike 
Connotea 
Del.icio.us What's this?
This article has been cited by other articles:
|
|
 |
|
  Joint Committee on Infant Hearing Year 2007 Position Statement: Principles and Guidelines for Early Hearing Detection and Intervention Programs Pediatrics, October 1, 2007; 120(4): 898 - 921. [Full Text] [PDF]
|
|
|
|
 |
|
 Y. Noguchi, K. Kurima, T. Makishima, M. H. de Angelis, H. Fuchs, G. Frolenkov, K. Kitamura, and A. J. Griffith Multiple Quantitative Trait Loci Modify Cochlear Hair Cell Degeneration in the Beethoven (Tmc1Bth) Mouse Model of Progressive Hearing Loss DFNA36 Genetics, August 1, 2006; 173(4): 2111 - 2119. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 R Varga, M R Avenarius, P M Kelley, B J Keats, C I Berlin, L J Hood, T G Morlet, S M Brashears, A Starr, E S Cohn, et al. OTOF mutations revealed by genetic analysis of hearing loss families including a potential temperature sensitive auditory neuropathy allele J. Med. Genet., July 1, 2006; 43(7): 576 - 581. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 F.-G. Zeng and S. Liu Speech perception in individuals with auditory neuropathy. J Speech Lang Hear Res, April 1, 2006; 49(2): 367 - 380. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 W. Tang, Y. Zhang, Q. Chang, S. Ahmad, I. Dahlke, H. Yi, P. Chen, D. L. Paul, and X. Lin Connexin29 Is Highly Expressed in Cochlear Schwann Cells, and It Is Required for the Normal Development and Function of the Auditory Nerve of Mice J. Neurosci., February 15, 2006; 26(7): 1991 - 1999. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
R. P. Wennberg, C. E. Ahlfors, V. K. Bhutani, L. H. Johnson, and S. M. Shapiro Toward Understanding Kernicterus: A Challenge to Improve the Management of Jaundiced Newborns Pediatrics, February 1, 2006; 117(2): 474 - 485. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. L. Berg, J. B. Spitzer, H. M. Towers, C. Bartosiewicz, and B. E. Diamond Newborn Hearing Screening in the NICU: Profile of Failed Auditory Brainstem Response/Passed Otoacoustic Emission Pediatrics, October 1, 2005; 116(4): 933 - 938. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 F.-G. Zeng, Y.-Y. Kong, H. J. Michalewski, and A. Starr Perceptual Consequences of Disrupted Auditory Nerve Activity J Neurophysiol, June 1, 2005; 93(6): 3050 - 3063. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 G. Rance and D. Aud Auditory Neuropathy/Dys-synchrony and Its Perceptual Consequences Trends in Amplification, March 1, 2005; 9(1): 1 - 43. [Abstract] [PDF]
|
|
|
|
|
|
T B Kim, B Isaacson, T A Sivakumaran, A Starr, B J B Keats, and M M Lesperance A gene responsible for autosomal dominant auditory neuropathy (AUNA1) maps to 13q14-21 J. Med. Genet., November 1, 2004; 41(11): 872 - 876. [Full Text] [PDF]
|
|
|
|
|
|
K. Stankovic, C. Rio, A. Xia, M. Sugawara, J. C. Adams, M. C. Liberman, and G. Corfas Survival of Adult Spiral Ganglion Neurons Requires erbB Receptor Signaling in the Inner Ear J. Neurosci., October 6, 2004; 24(40): 8651 - 8661. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 B Ceranic and L M Luxon Progressive auditory neuropathy in patients with Leber's hereditary optic neuropathy J. Neurol. Neurosurg. Psychiatry, April 1, 2004; 75(4): 626 - 630. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
F.-G. Zeng Trends in Cochlear Implants Trends in Amplification, March 1, 2004; 8(1): 1 - 34. [Abstract] [PDF]
|
|
|
|
 |
|
 A. Starr, H. J. Michalewski, F.-G. Zeng, S. Fujikawa-Brooks, F. Linthicum, C. S. Kim, D. Winnier, and B. Keats Pathology and physiology of auditory neuropathy with a novel mutation in the MPZ gene (Tyr145->Ser) Brain, July 1, 2003; 126(7): 1604 - 1619. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M. Aldosari, A. Mabie, and A. M. Husain Delayed Visual Maturation Associated With Auditory Neuropathy/Dyssynchrony J Child Neurol, May 1, 2003; 18(5): 358 - 361. [Abstract] [PDF]
|
|
|
|
|
|
R Varga, P M Kelley, B J Keats, A Starr, S M Leal, E Cohn, and W J Kimberling Non-syndromic recessive auditory neuropathy is the result of mutations in the otoferlin (OTOF) gene J. Med. Genet., January 1, 2003; 40(1): 45 - 50. [Full Text] [PDF]
|
|
|
|
 |
|
 C Dunkley, A Farnsworth, S Mason, M Dodd, and K Gibbin Screening and follow up assessment in three cases of auditory neuropathy Arch. Dis. Child., January 1, 2003; 88(1): 25 - 26. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 C. Madden, M. Rutter, L. Hilbert, J. H. Greinwald Jr, and D. I. Choo Clinical and Audiological Features in Auditory Neuropathy Arch Otolaryngol Head Neck Surg, September 1, 2002; 128(9): 1026 - 1030. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. Podwall, D. Podwall, T. G. Gordon, P. Lamendola, and A. P. Gold Unilateral Auditory Neuropathy: Case Study J Child Neurol, April 1, 2002; 17(4): 306 - 309. [Abstract] [PDF]
|
|
|
|
|
|
S. Fujikawa and A. Starr Vestibular Neuropathy Accompanying Auditory and Peripheral Neuropathies Arch Otolaryngol Head Neck Surg, December 1, 2000; 126(12): 1453 - 1456. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
Joint Committee on Infant Hearing, American Academy of Audiology, American Academy of Pediatrics, American Speech-Language-Hearing Association, and Directors of Speech and Hearing Programs in State Year 2000 Position Statement: Principles and Guidelines for Early Hearing Detection and Intervention Programs Pediatrics, October 1, 2000; 106(4): 798 - 817. [Full Text] [PDF]
|
|
|
|
 |
|
 B. J. B. Keats and C. I. Berlin Genomics and Hearing Impairment Genome Res., January 1, 1999; 9(1): 7 - 16. [Abstract] [Full Text]
|
|