Measuring and analyzing of otoacoustic emission tests of a family with genetic progressive sensorineural hearing loss

Objetive:To study the diagnostic significance of otoacoustic emission in patients with genetic progressive sensorineural earing loss.Method:To analyse the cochlear function pure tone andiometry Transied Evoked Otoacoustic Emission (TEOAE) and distortion Products Otoacoustic Emission (DPOAE) were measured in 54 members of a six generation family with genetic progresive sensorinearal 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.