Use and Benefits of Bone-Anchored Hearing Aids in Goldenhar Syndrome: A Case Report

Introduction: The Goldenhar Syndrome may cause hearing damages as a result of improper development of the outer, middle, and inner ear. In patients with conductive or mixed hearing loss, the Bone-anchored hearing aid (BAHA) 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).

Objectives: To evaluate the benefit of the hearing aid used by a patient with Goldenhar Syndrome as treatment, through the result of the functional gain test and also the satisfaction provided by it, according to the SADL questionnaire.

Resumed report: D.C.S., 35 years old, with bilateral mixed hearing loss and bilateral BAHA's user. The functional gain exam certificate was analyzed and the Brazilian SADL questionnaire was applied. 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 (the maximum score is 7, that indicates total satisfaction).

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.

Keywords: Goldenhar Syndrome, hearing loss, patient satisfaction, hearing aids.