Table 1.
Prevalence of hearing loss in 58 Dutch adults with Pompe disease compared to the general population, stratified by age
| Dutch adult Pompe patients | General population (Davis 1989; Johansson and Arlinger 2003; Wilson et al. 1999) | |||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Better-hearing ear** | Worse-hearing ear*** | Better-hearing ear** | Worse-hearing ear*** | |||||||||||
| Age group | n | ≥ 25–45 dB* | ≥ 45–65 dB | ≥ 65 dB | ≥ 25–45 dB | ≥ 45–65 dB | ≥ 65 dB | n | ≥ 25–45 dB | ≥ 45–65 dB | ≥ 65 dB | ≥ 25–45 dB | ≥ 45–65 dB | ≥ 65 dB |
| 20-50 yrs | 25 | 1 (4%) | 1 (4%) | - | 3 (12%) | 1 (4%) | - | 1869 | 1.6-3.6% | 0-0.8% | 0-0.3% | 5.2-10.6% | 0-2.8% | 0-0.8% |
| 51-60 yrs | 17 | 2 (12%) | 1 (6%) | - | 6 (35%) | 1 (6%) | - | 1000 | 11.3-18.9% | 0.7-4.0% | 0-0.9% | 22.6-33.8% | 2.8-12.2% | 0.6-5.2% |
| 61-70 yrs | 13 | 4 (31%) | - | - | 5 (38%) | 1 (8%) | - | 810 | 36.8-48.3% | 2.5-7.4% | 0-2.3% | 51.2-61.3% | 10.4-19.0% | 1.9-7.5% |
| 71-80 yrs | 3 | 2 (67%) | - | 1 (33%) | 2 (67%) | - | 1 (33%) | 542 | 60.3-73.8% | 17.6-51.6% | 1.8-3.3% | 71.6-86.0% | 33.2-41.2% | 6.3-12.6% |
| Overall | 58 | 9 (16%) | 2 (3%) | 1 (2%) | 16 (28%) | 3 (5%) | 1 (2%) | 4224 | 16.1-16.9 % | 2.8-3.9% | 0.2-1.1% | 22.2-26.3% | 6.2-9.3% | 1.0-3.5% |
*The hearing impairment as reported in this table is graded according to the WHO guidelines. It is measured as dB (decibel) hearing level averaged over 0.5, 1.2 and 4 kHz: 25–45 dB signifies slight hearing loss, 45–65 dB signifies moderate hearing loss, and >65 dB signifies profound hearing loss. ** These columns report the hearing loss in the better-hearing ear of patients with bi-lateral hearing loss. *** These columns report the hearing loss in the worse-hearing ear of patients with either bi-lateral (12 of the 20 patients) or uni-lateral hearing loss (8 of the 20 patients)