Table 2

 Summary of audiological data

NSRAN individualAge testedAudiometric lossAudiogram shapeABRCMOAEAcoustic reflexesDiagnosis
*Absent bilaterally; †present bilaterally; ‡not tested. ABR, auditory brainstem response; CM, cochlear microphonics;OAE, otoacoustic emissions.
3456-13 yearsProfoundCorner–*+†+NT‡NSRAN
3456-215 monthsProfoundCorner++NTNSRAN
3466-14 yearsProfoundFlatNI§+NINSRAN
3466-216 monthsProfoundFlatNI+NINSRAN
3467-1 37°C6 yearsMild to normalRisingAbnormal++TS-NSRAN
3467-1 37.8°CMild to moderateCookie biteNTNTNTNTTS-NSRAN
3467-1 38.1°CR: profound, L: severe to profoundR: flat, L: risingNT+TS-NSRAN
3467-2 37°C2 yearsmild to normalRisingAbnormal++TS-NSRAN
3539-16 yearsModerate to severeRising++NSRAN
3539-23 yearsModerate to severeRising++NSRAN
3540-13 yearsNTNT++NSRAN
53510-126 yearsProfoundCornerNTNTNTNSRHL
53510-233 yearsSevere to profoundCornerNTNTNTNTNSRHL
53510-335 yearsProfoundCornerNTNTNTNTNSRHL