The syndrome of Kearns-Sayre (SKS) is a rare medical condition. One is about a mitochondrial citopaty that uses to attend a course with gradual external ophthalmolplegy, retinopathy to pigment and blockade of cardiac conduction. Its pathophysiology involves multiple only in the mitochondrial DNA, spontaneous deletions, in ovum or zygote (not inherited), with manifestations before the 20 years. It can attack multiple agencies and generate diverse symptoms as hypoacusia and anacusis. The development of deafness in the SKS is of otolaryngology and recognized interest for gradual bilateral hypoacusia, of sensorineural character. In the service of otolaryngology of the HC/UFMG, we receive patient carrying from this syndrome. V.A.G.P, 14 years, masculine, looked the service in which had November of 2010 diagnostic one of hypoacusia and for evaluation of the use of auditory device of sonorous amplification (AASI). The syndrome was diagnosised in 2003 in the ophthalmolplegy validity, degeneration to pigment of the retina, hyperparathyroidism and tubular renal acidosis. Patient evolved until the present date with diverse occurrences. The blockade to atrioventriculary, important for the diagnosis of the SKS, was confirmed in electrophysiologist study with catheterization carried through in March of 2010. Beyond being followed by diverse medical specialties, the patient carries through diverse audiometry in order to observe the progression of the auditory deficit. Audiometry carried through to the 5, 7, 11 and 14 years show the gradual evolution of the sensorineural, bilateral hearing loss. The picture of this shows patient with the typical presentation of the hearing loss in the described SKS in literature. It strengthens the importance of the otolaryngologic accompaniment how much to the evaluation of the use of the AASI in the therapeutical one, and consequently, in the improvement of its quality of life.