Worse-hearing ear pure-tone thresholds plotted against the percentiles obtained from age-matched normative data (ISO 7029) and the criterion for clinically normal hearing (20 dB hearing loss) for 0.5, 1, 2, 4 and 8 kHz. Neurological status, xeroderma pigmentosum-type neurological degeneration, patients with xeroderma pigmentosum (XP) without neurological involvement, and non-xeroderma pigmentosum type neurological involvement, is indicated by colour-coding. HL = hearing loss.