Perfil audiológico e utilização e benefício de dispositivo de amplificação sonora na síndrome de Goldenhar: um relato de caso Audiological profile and the use and benefits of hearing aids in Goldenhar syndrome: a case report

Background: The Goldenhar Syndrome may cause hearing damages as a result of improper development of the outer, middle and inner ear. A hearing loss can be categorized in three different ways: conductive, mixed or sensorineural, depending on the location of the malformation. In patients with conductive or mixed hearing loss, the BAHA® (Bone-anchored hearing aid) is a good hearing aid option. In order to evaluate the satisfaction provided by this hearing aid, functional gain testing is not enough. The level of satisfaction with a hearing aid used on a daily basis can be studied through the questionnaire Satisfaction with Amplification in Daily Life – SADL. Objective: To outline the audiologic profile, through the analysis of audiometric results of a patient with Goldenhar Syndorme, as well as to evaluate the benefit of the hearing aid used by her as treatment, through the result of the functional gain test and also the satisfaction provided by it, according to the Brazilian SADL questionnaire. Methodology: The audiometric and functional gain exam certificates were analyzed and the Brazilian SADL questionnaire was applied. Results: The air-conduction thresholds did not change much as the bone-conduction thresholds presented larger changes, with a 30 dB average bilaterally. The functional gain showed a 25 dB average with the BAHA in the left ear, another 30 dB average with the BAHA in the right ear and a 25 dB average with the BAHA being used bilaterally. With the SADL questionnaire, the global score average was 6,06. Conclusion: This study showed that the bone-conduction hearing aids were beneficial for this patient, for offering an audibility improvement and a high level of satisfaction with their use.