Hearing Impairment In Oculoauriculovertebral Dysplasia (Goldenhar Syndrome): Literature Review

Abstract

This paper reviews the literature on patients with oculoauriculovertebral dysplasia (OAVD, Goldenhar syndrome) in the context of methods to improve their hearing, an area of interest to otorhinolaryngologists, audiologists, and phoniatrists. The papers reviewed describe individual cases and groups of patients diagnosed with the syndrome. They are of interest due to the range of therapeutic approaches used and the percentage of favourable results. The available literature is sparse, particularly in terms of improving hearing in OAVD. In these patients, degrees of hearing loss or deafness are major problems resulting from complex external, middle, and inner ear defects, which can also lead to speech development disorders. There is therefore a need for a closer look at the syndrome, including how to identify patients, indications for surgical treatment, use of other methods to improve hearing, and methods of rehabilitation.