Introduction:

Goldenhar’s syndrome is a congenital polymalformative syndrome that is evident at birth. It is a malformation of the facial-auriculo-vertebral spectrum, which has associated auricular anomalies in the sections of the auditory canal, causing bilateral mixed hearing loss in about one third of the cases. For its satisfactory rehabilitation it requires the participation of a multidisciplinary team, among them: pediatricians, audiologists, plastic surgeons, ophthalmologists and speech-language therapists.

Case report:

a 3-year old and 10 months preschooler, who was evaluated at the Speech Therapy Laboratory of Psychomotor Skills and Early Stimulation at Pinar del Rio University at one year old for presenting delay of language development. A pediatric speech-language clinical history was written, including positive physical examination data such as severe bilateral microtia grade IV in the right ear and almost anotia in the left ear, hemi-facial microsomia, left unilateral macrostomia with deviation of the labial commissure to the left and appendiceal appendages in the path of the first and second branchial arch. The auditory study determined moderate to severe mixed sensorineural hearing loss in the right ear and profound sensorineural hearing loss in the left ear.

Conclusions:

children from early infancy with hearing loss associated with Goldenhar´s syndrome have a successful evolution, taking into account the earliest possible diagnosis and intervention. The study allowed the description of a case with hearing loss associated with Goldenhar´s syndrome, which revealed a good development of expressive and comprehensive language.

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