[Measuring and analyzing of otoacoustic emission tests of a family with genetic progressive sensorineural hearing loss].
OBJECTIVE
To study the diagnostic significance of otoacoustic emission in patients with genetic progressive sensorineural hearing loss.


METHODS
To analyse the cochlear function pure tone audiometry Transient Evoked Otoacoustic Emission (TEOAE) and distortion Products Otoacoustic Emission (DPOAE) were measured in 54 members of a six-generation family with genetic progressive sensorineural hearing loss (108 cases) and 50 normal people.


RESULTS
The family was autosomal dominant heritage. In the group with pure tone thresholds more than 20 dB HL, the amplitudes of TEOAE descended or disappeared in 84% cases and the amplitudes of DPOAE declined or vanished in 82% cases. In the group with normal pure tone thresholds, the amplitudes of TEOAE descended in 75% cases and the amplitudes at one of the frequencies of DPOAE dropped or disappeared in 64% cases. But the incidence of TEOAE and DPOAE in normal people was 100%.


CONCLUSIONS
OAE had important significance in the diagnosis of genetic hearing loss at early stage.
Yu
H
H
Department of Otolaryngology, People's Hospital of Beijing Medical University, Beijing 100044.
Ke
X M
XM
Yu
D L
DL
Li
Q
Q
Liu
Y H
YH
Ding
G Y
GY
D000293:Adolescent; D000328:Adult; D001301:Audiometry, Pure-Tone; D002648:Child; D003051:Cochlea / Q000503:physiopathology*; D005260:Female; D006319:Hearing Loss, Sensorineural / Q000175:diagnosis / Q000235:genetics*; D006801:Humans; D008297:Male; D008875:Middle Aged; D017084:Otoacoustic Emissions, Spontaneous; D010375:Pedigree