Table 3.
Demographics and hearing loss characteristics in sickle cell patients with sensorineural hearing loss.
| Parameter | Number of Patients (n =12) (%) | Mean ± SD (Range) |
|---|---|---|
| Sex (male) | 7 (58.3%) | |
| Mean age at first audiogram (years) | 12 | 8.8 ± 6.1 (0.9–17.6) |
| Genotype HbSS | 10 (83.3%) | |
| Genotype HbSC | 2 (16.7%) | |
| History of cerebrovascular disease | 3 (25%) | |
| History of abnormal transcranial Doppler screen | 3 (25%) | |
| R. Ear Average Pure Tone Average (dB HL):a | 10 | 43.3 ± 28.9 (13.8–100) |
| Normal hearing (< 20 dB HL) | 2 (20%) | |
| Mild hearing loss (20–40 dB HL) | 4 (40%) | |
| Moderate hearing loss (40–60 dB HL) | 1 (10%) | |
| Severe hearing loss (60–80 dB HL) | 2 (20%) | |
| Profound hearing loss (> 80 dB HL) | 1 (10%) | |
| L. Ear Average Pure Tone Average (dB HL):a | 10 | 40.8 ± 29.7 (8.8–100) |
| Normal hearing (< 20 dB HL) | 3 (30%) | |
| Mild hearing loss (20–40 dB HL) | 3 (30%) | |
| Moderate hearing loss (40–60 dB HL) | 2 (20%) | |
| Severe hearing loss (60–80 dB HL) | 1 (10%) | |
| Profound hearing loss (> 80 dB HL) | 1 (10%) | |
| Absent DPOAEsb | 7 of 9 tested (77.8%) |
a
Pure tone averages could not be calculated in two patients with SNHL, as at least one hearing frequency was missing from 500 to 2000 Hz.
b
Counted as absent, if absent at any range within the tested range, suggestive of abnormal outer hair cell function.