Cochlear receptor (microphonic and summating potentials, otoacoustic emissions) and auditory pathway (auditory brain stem potentials) activity in auditory neuropathy.
OBJECTIVE
To define both auditory nerve and cochlear receptor functions in subjects with auditory neuropathy (AN).


METHODS
We tested 33 AN subjects (66 ears) and compared them with 21 healthy subjects (28 ears). In AN subjects, the average pure-tone (1, 2, and 4 kHz) threshold loss was 57 dB HL. Click stimuli were used to elicit transient evoked otoacoustic emissions (TEOAEs), cochlear microphonics (CMs), and auditory brain stem responses (ABRs). Both cochlear and ABR potentials were recorded from surface electrodes (vertex-ipsilateral mastoid) using averaging procedures. The amplitudes and latencies of CMs and ABRs and the amplitude of the TEOAEs were analyzed.


RESULTS
CM amplitudes recorded from normal ears decreased as a function of subject age. CMs recorded from AN subjects fell within the normal age-adjusted range in 60% of the subjects and were >2 SEEs (standard error of estimate) above the age-adjusted normal regression in 40% of the subjects. TEOAEs were absent in 19 (30%) AN ears (bilaterally in eight, and unilaterally in three subjects) and were present in 44 ears. In AN subjects, correlations among CM amplitude, TEOAE amplitude, and pure-tone average thresholds were not significantly related. CM amplitudes were not significantly different whether TEOAEs or ABRs were present or absent. The ABR was present in 21% of AN subjects and consisted of a low-amplitude Wave V without a preceding Wave I. Measures of CM amplitude and PTA hearing loss were not significantly different in those AN ears with a preserved ABR compared with ears with absent ABRs. Summating potentials to transient click stimuli were of small amplitude (<0.1 microV) and detectable in approximately 50% of the AN and healthy control subjects limiting formal analysis of summating potentials.


CONCLUSIONS
In a significant proportion of AN subjects, we found abnormalities of cochlear receptor function, including elevated CM amplitudes and absence of TEOAEs. These two abnormalities occurred independently of each other. A low amplitude Wave V of the ABR was found in approximately one-fifth of AN subjects, evidence that neural synchrony can be partially preserved in some subjects with this disorder.
Starr
A
A
Department of Neurology, University California, Irvine 92697-4290, USA.
Sininger
Y
Y
Nguyen
T
T
Michalewski
H J
HJ
Oba
S
S
Abdala
C
C
D000293:Adolescent; D000328:Adult; D001301:Audiometry, Pure-Tone; D001306:Auditory Pathways / Q000503:physiopathology*; D002648:Child; D002675:Child, Preschool; D003051:Cochlea / Q000503:physiopathology*; D005071:Evoked Potentials / Q000502:physiology*; D016057:Evoked Potentials, Auditory, Brain Stem / Q000502:physiology*; D005260:Female; D006319:Hearing Loss, Sensorineural / Q000175:diagnosis / Q000503:physiopathology*; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D017084:Otoacoustic Emissions, Spontaneous / Q000502:physiology*; D012720:Severity of Illness Index