How hidden is hidden hearing loss? Self-reported listening problems in charcot Marie tooth disease

Introduction

Laboratory studies have revealed hidden hearing loss in patients with Charcot-Marie-Tooth (CMT) disease, the most prevalent inherited neuropathy, which may impact their quality of life. The current study distinguished between CMT type 1, which involves demyelination of the peripheral nerves, and type 2, which concerns dysfunction of peripheral nerves due to axonopathy. The self-reported effects were investigated of CMT1 and CMT2 on listening problems and related social and attentional problems in everyday communicative situations.

Methods

Data were collected from 42 CMT1 patients, 30 CMT2 patients, and 72 matched controls (selected from 135 control-group participants), who completed questionnaires concerning listening in everyday and noisy situations, social problems due to hearing difficulties, and problems regarding auditory sustained attention.

Results

CMT2 patients seemed to suggest only minor listening difficulties. No effects were found for social and attentional problems. In addition, CMT1 patients were found to be associated with more overall listening difficulties in everyday situations, compared to controls. CMT1 patients reported substantial listening difficulties, particularly in noisy and reverberant environments. These problems might be caused by underlying binaural hearing problems combined with reduced processing of temporal and acoustic information, as suggested in the literature. These listening difficulties were associated with social problems, such as social insecurity or even social avoidance, and problems regarding auditory sustained attention.

Conclusions

These findings strongly suggest that CMT1 impacts quality of life concerning social communication. The findings presented in this study will benefit diagnosis and treatment of hearing difficulties and related problems in CMT1 patients.