This proposal will study a recently recognized form of hearing disorder called auditory neuropathy (AN). Different from cochlear damage, AN is characterized by normal measures of cochlear outer hair cells but abnormal measures of the central auditory pathway beginning with auditory nerve. The hearing disorder typically affects speech comprehension out-of-proportion to the pure tone loss, particularly speech recognition in noise. AN is not rare and accounts for 10 percent of newborns identified as having hearing loss. The disorder occurs in children and adults. In adults the disorder is commonly associated with a peripheral neuropathy. Loss of neural synchrony is proposed as a cardinal mechanism underlying the hearing disorder. The sites of abnormality in AN could include auditory nerve and/or inner hair cells and their synapses with auditory nerve dendrites. Our long-term goals are to understand the AN mechanisms and functions in order to provide a scientific basis for alleviating the hearing deficit in AN subjects. We propose three experiments using both psychophysical and elecrophysiological techniques to characterize: (1) fundamental and complex auditory processes in AN subjects in order to explain their speech recognition difficulty, particularly in noise; (2) distinguish between the site of the disorder as being at auditory nerve or at inner hair cell/synapse complex; and (3) optimize signal processing for AN subjects with cochlear implants. The resultsof our studies could have major impact on the diagnosis, classification, and treatment of auditory neuropathy.

Agency
National Institute of Health (NIH)
Institute
National Institute on Deafness and Other Communication Disorders (NIDCD)
Type
Research Project (R01)
Project #
2R01DC002618-04A2
Application #
6434339
Study Section
Special Emphasis Panel (ZRG1-IFCN-5 (08))
Program Officer
Luethke, Lynn E
Project Start
1996-09-01
Project End
2005-02-28
Budget Start
2002-03-01
Budget End
2003-02-28
Support Year
4
Fiscal Year
2002
Total Cost
$343,829
Indirect Cost
Name
University of California Irvine
Department
Neurology
Type
Schools of Medicine
DUNS #
161202122
City
Irvine
State
CA
Country
United States
Zip Code
92697
Habibi, Assal; Wirantana, Vinthia; Starr, Arnold (2014) Cortical Activity during Perception of Musical Rhythm; Comparing Musicians and Non-musicians. Psychomusicology 24:125-135
Dimitrijevic, Andrew; Pratt, Hillel; Starr, Arnold (2013) Auditory cortical activity in normal hearing subjects to consonant vowels presented in quiet and in noise. Clin Neurophysiol 124:1204-15
Pratt, Hillel; Abbasi, Dalal Abu-Amneh; Bleich, Naomi et al. (2013) Spatiotemporal distribution of cortical processing of first and second languages in bilinguals. II. Effects of phonologic and semantic priming. Hum Brain Mapp 34:2882-98
Wynne, Dwight P; Zeng, Fan-Gang; Bhatt, Shrutee et al. (2013) Loudness adaptation accompanying ribbon synapse and auditory nerve disorders. Brain 136:1626-38
Pratt, Hillel; Abbasi, Dalal Abu-Amneh; Bleich, Naomi et al. (2013) Spatiotemporal distribution of cortical processing of first and second languages in bilinguals. I. Effects of proficiency and linguistic setting. Hum Brain Mapp 34:2863-81
Dimitrijevic, Andrew; Starr, Arnold; Bhatt, Shrutee et al. (2011) Auditory cortical N100 in pre- and post-synaptic auditory neuropathy to frequency or intensity changes of continuous tones. Clin Neurophysiol 122:594-604
Pratt, Hillel; Starr, Arnold; Michalewski, Henry J et al. (2010) A comparison of auditory evoked potentials to acoustic beats and to binaural beats. Hear Res 262:34-44
Santarelli, Rosamaria; Del Castillo, Ignacio; Rodriguez-Ballesteros, Montserrat et al. (2009) Abnormal cochlear potentials from deaf patients with mutations in the otoferlin gene. J Assoc Res Otolaryngol 10:545-56
Pratt, Hillel; Starr, Arnold; Michalewski, Henry J et al. (2009) Auditory-evoked potentials to frequency increase and decrease of high- and low-frequency tones. Clin Neurophysiol 120:360-73
Pratt, Hillel; Starr, Arnold; Michalewski, Henry J et al. (2009) Cortical evoked potentials to an auditory illusion: binaural beats. Clin Neurophysiol 120:1514-24

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