Auditory abnormalities, including 'precocious presbyacusis', in myotonic dystrophy.

Auditory function tests were performed on 13 patients with myotonic dystrophy (MD). Seven patients had a sensorineural high-frequency hearing loss (HFL) of 30-85 dB at 8 kHz in their pure-tone audiogram, which was in excess of that expected for their age and could be attributed to MD. Their hearing loss resembled 'precocious presbyacusis', i.e. if the patients had been considerably older (or 'functionally' older) than they really were, their HFL could (to some extent) have been attributed to presbyacusis alone. The HFL showed the phenomenon of (genetic) anticipation. Tympanograms and acoustic reflexes were normal. Brainstem auditory evoked potentials (BAEPs) showed a significant increase in the I-V interpeak interval (by 0.35-0.7 ms) and in the III-V interpeak interval (by 0.21-0.67 ms). There was no correlation between the BAEP and the audiometric findings. It should be noted that precocious presbyacusis may be linked to specific gene defects.