title: [Optic atrophy, sensorineural deafness and sensory neuropathy].
abstract: We present a patient with slowly progressing optic atrophy, sensorineural deafness and sensory neuropathy. Clinical examination and testing revealed the exclusively sensorineural nature of this syndrome. Nerve biopsy pointed to primary degeneration. Our review of the literature indicates that this syndrome is categorized as heredo-degenerative.
authors.affiliation: Sección de Neurología, Hospital General de Elche, Valencia.
authors.forename: J A
authors.initials: JA
authors.lastname: Monge Argiles
authors.forename: J
authors.initials: J
authors.lastname: Alom Poveda
authors.forename: J
authors.initials: J
authors.lastname: Espinosa Martínez
authors.forename: M
authors.initials: M
authors.lastname: Pérez Arroyo
authors.forename: M J
authors.initials: MJ
authors.lastname: Roca Estelles
mesh_terms: D000293:Adolescent; D001299:Audiometry; D001369:Axons / Q000473:pathology; D005260:Female; D006319:Hearing Loss, Sensorineural / Q000150:complications* / Q000175:diagnosis; D015417:Hereditary Sensory and Motor Neuropathy / Q000175:diagnosis / Q000473:pathology*; D006801:Humans; D009896:Optic Atrophy / Q000150:complications / Q000175:diagnosis / Q000473:pathology*; D013497:Sural Nerve / Q000503:physiopathology / Q000648:ultrastructure
vernacular_title: Atrofia óptica, sordera neurosensorial y neuropatía sensitiva.