The role of auditory brainstem response in diagnosing auditory impairments of Dejerine-Sottas

Abstract Dejerine-Sottas disease is classified as hereditary motor sensory neuropathy (HMSN) type III and shows evidence of Friedreich's ataxia, significant reduction in nerve conduction velocity (NCV), hypomyelination and demyelination of the nerve fibers. In this study, a 10-years-old girl with Dejerine-Sottas disease is presented in which routine clinical signs (ataxia and reduced NCV) seem with significant impairments of auditory brainstem pathway. It is indicated that pure tone audiometry, standard tympanometry (tympanometry and acoustic reflex), and otoacoustic emissions (OAEs) showed normal results in both ears (normal peripheral auditory system). In contrast, auditory brainstem responses (ABRs) indicated abnormal findings in absolute latencies of I, III, and V and inter-peak latencies of I–III and I–V. These findings suggested auditory brainstem involvement especially in low and mid regions.