title: [Kearns-Sayre syndrome from the otorhinolaryngologic viewpoint].
abstract: External ophthalmoplegia, retinal pigmentary degeneration and heart block constitute the trias of Kearns-Sayre's syndrome. The aetiology of this disorder of oxidative metabolism is unknown. This syndrome must be more frequent than described. In the ENT field there is an extensive lack of differentiated data. Central neural and peripheral hearing disorders and vestibular disorders are in fact significant, as are also dysphagia, hoarseness and dysarthria in consequence of central and peripheral disorders in muscular function. The authors report on ENT findings in 4 patients with verified Kearns-Sayre's syndrome. Progression of central disorders enhances an unfavourable prognosis. Histochemical, biochemical and electron microscopic data are still lacking for the proper grading and assessment of clinical findings.
mesh_terms: D000293:Adolescent; D000328:Adult; D001301:Audiometry, Pure-Tone; D002648:Child; D002675:Child, Preschool; D005260:Female; D006313:Hearing Loss, Central / Q000175:diagnosis; D006319:Hearing Loss, Sensorineural / Q000175:diagnosis; D006801:Humans; D007223:Infant; D007625:Kearns-Sayre Syndrome / Q000175:diagnosis*; D007759:Labyrinth Diseases / Q000175:diagnosis; D008297:Male; D009886:Ophthalmoplegia / Q000175:diagnosis*; D010038:Otorhinolaryngologic Diseases / Q000175:diagnosis*; D012022:Reflex, Acoustic; D014724:Vestibular Function Tests; D014722:Vestibule, Labyrinth / Q000503:physiopathology
vernacular_title: Das Kearns-Sayre-Syndrom aus hals-nasenohrenärztlicher Sicht.