Audiological Findings in Charcot–Marie–Tooth Disease Type 4C | Zendy

Rafael Sivera | Zendy; Laura Cavallé | Zendy; Juan J. Vílchez | Zendy; Carmen Espinós | Zendy; Herminio Pérez Garrigues | Zendy; Teresa Sevilla | Zendy

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Audiological Findings in Charcot–Marie–Tooth Disease Type 4C

Author(s) -

Rafael Sivera,

Laura Cavallé,

Juan J. Vílchez,

Carmen Espinós,

Herminio Pérez Garrigues,

Teresa Sevilla

Publication year - 2017

Publication title -

the journal of international advanced otology

Language(s) - English

Resource type - Journals

SCImago Journal Rank - 0.518

H-Index - 13

eISSN - 2148-3817

pISSN - 1308-7649

DOI - 10.5152/iao.2017.3379

Subject(s) - medicine , auditory neuropathy , audiology , hearing loss , acoustic reflex , audiometry , abnormality , vestibular evoked myogenic potential , brainstem , vestibular system , cochlear nerve , cochlea , psychiatry

Charcot-Marie-Tooth disease type 4C (CMT4C) is a hereditary demyelinating early onset neuropathy with prominent unsteadiness and occasional cranial nerve involvement. Vestibulopathy caused by the dysfunction of cranial nerve VIII has been demonstrated in a high percentage of these patients, but the presence and degree of auditory neuropathy are unknown. The aim of the study was to characterize the hearing abnormalities of a series of patients with CMT4C and to determine the presence and severity of auditory neuropathy (AN) in these patients.

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