Table 1.
Features of subjects with auditory neuropathy
| Subject | Site of auditory neuropathy | Age (years) | Gender | Ear tested | OAEs | ABR Wave V latency | PTA | Gap (ms) | Gene | Special features | |
|---|---|---|---|---|---|---|---|---|---|---|---|
| (Low) (dBHL) | (High) (dBHL) | ||||||||||
| AN30 | Synapse | 24 | M | Left | Yes | 7.1 | 8 | 2 | 5.8 | OTOF | Temperature sensitive |
| Right | Yes | 6.9 | 12 | 7 | |||||||
| AN31 | Synapse | 9 | M | Left | Yes | Absent | 20 | 8 | 15 | OTOF | Temperature sensitive |
| Right | Yes | 7 | 23 | 17 | 15 | ||||||
| AN32 | Synapse | 18 | F | Left | Yes | 7.5 | 28 | 40 | 12 | OTOF | Temperature sensitive |
| AN33 | Synapse | 13 | M | Left | Yes | 7.6 | 17 | 10 | 11 | OTOF | Temperature sensitive |
| AN2 | Nerve | 65 | F | Left | Yes | Absent | 77 | DNT | 15 | MPZ | CMT neuropathy |
| Right | Yes | 85 | 52 | 9 | |||||||
| AN3 | Nerve | 30 | F | Right | No | Absent | 83 | 90 | 130 | ? | Neuropathy |
| AN13 | Nerve | 37 | F | Left | Yes | Absent | 52 | 27 | 8 | ? | Peripheral neuropathy |
| AN34 | Nerve | 15 | F | Left | No | Absent | 28 | 35 | DNT | FX | FRDA neuropathy |
| Right | No | Absent | 27 | 27 | |||||||
| AN36 | Nerve | 56 | F | Left | Yes | 6.2 | 62 | 18 | 10 | ? | Neuropathy |
| Right | Yes | Absent | 47 | 30 | 10 | ||||||
| AN40 | Nerve | 14 | M | Right | Yes | Absent | 50 | 35 | 11 | ? | Optic neuropathy |
| All AN subjects | 10 | 9–65 | 6F 4M | Both | 7/10 | 7.1 | 42 | 35 | 23.9 | ||
AN = auditory neuropathy; CMT = Charcot–Marie–Tooth; dBHL = decibels hearing level; DNT = did not test; FRDA = Friedreich’s ataxia; Nerve = neural auditory neuropathy; OAE = otoacoustic emission; PTA (high) = pure tone threshold average at 4.0, 6.0, 8.0 kHz; PTA (low) = pure tone threshold average at 0.5, 1.0, 2.0 kHz; Synapse = ribbon synapse disorder; ? = unknown.