While clinical studies suggest that patients with Charcot-Marie-Tooth (CMT) disease may suffer from ‘hidden’ deafness, a recent Dutch study used self-questionnaires to assess the hearing difficulties of these patients and their repercussions in everyday situations.
- The study included 42 patients with CMT1, 30 with CMT2 and 72 matched controls.
- Patients with CMT1 were found to have more difficulty listening on a daily basis than controls, in noisy environments and those with acoustic reverberation (theatres, etc.).
- Social difficulties linked to these hearing problems significantly affected only the men in this cohort, with feelings of insecurity and social avoidance.
- Difficulties in maintaining sustained attention were also reported.
- The problems experienced by CMT1 patients could be due to demyelination of the auditory nerves.
- Patients with CMT2 reported minor listening difficulties with no effect on social behaviour or attention.
Raising awareness of the hidden deafness of CMT1 patients could help audiology professionals to make the correct diagnosis and implement appropriate treatment. In particular, auditory training programmes could improve speech perception in noisy conditions.
