[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. METHOD: 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. RESULT: 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%. CONCLUSION: OAE had important significance in the diagnosis of genetic hearing loss at early stage.