Introduction: Hearing loss may be related to several factors, among them is hearing loss due to certain genetic syndromes. The Kearns-Sayre syndrome is characterized by mutations in mitochondrial DNA, which is responsible for the production of energy (adenosine triphosphate), which is of utmost importance for the development of structures requiring this energy, as the cochlea.

Objective: To describe the medical evaluation results, the audiological diagnosis and intervention of two twin sisters, diagnosed with Kearns- Sayre syndrome and hearing loss.

Resumed Report: This case was accompanied at the hospital since 2000, due to the progressive characteristic of hearing loss found by audiological tests, and the findings in cases related to the syndrome. The intervention with the hearing aids has become not much beneficial to good oral communication for case 1, who happened to have the diagnosis of bilateral profound hearing loss. So after discussions in clinical meetings, the team opted for the indication of cochlear implants for this patient (case 1), achieving good results. Since her twin sister (case 2) has had good results with hearing aids, the patient (case 2) will continue the audiologic follow up, to enable the verification of the developments in the case and the discussion of a new approach if necessary.

Conclusion: Patients suspected or diagnosed with Kearns-Sayre syndrome should seek audiological diagnosis, since it may be a possible progressive hearing loss, which requires rehabilitation with the use of hearing devices.