Hidden hearing loss in a hereditary peripheral neuropathy: evidence from a mouse model of Charcot Marie Tooth type 1A.

Hidden Hearing Loss (HHL), an auditory disorder estimated to affect 12-15% of the adult population, is believed to impact auditory processing and hearing clarity in individuals with normal audiometric thresholds. Based on animal studies, the loss of inner hair cell synapses is currently considered its primary cause. Here we provide evidence that mild disruptions of auditory nerve myelination, i.e., disorganization of auditory nerve heminodes, can also cause HHL. Our results suggest that patients suffering from Charcot-Marie-Tooth type 1A, the most common hereditary peripheral neuropathy, are likely to experience HHL. Studies of hearing in CMT1A patients might thus help in the development of robust clinical tests for HHL, which are currently lacking.