Normalization of auditory brainstem response in infant with Cornelia de Lange syndrome

Abstract

  Cornelia de Lange syndrome is a congenital disease that was first described by the Dutch pediatrician Cornelia de Lange. This syndrome is a multiple malformation disorder characterized by specific facial features, abnormalities of the limbs, mental retardation, and growth delay. It is also known to involve hearing impairment, but reports are scarce. We report the case of a 1-month-old infant suffering from this syndrome who was suspected of having a moderate hearing loss. His hearing has improved during the follow-up examination. When he was 4 years and 1 month old, his auditory brain stem response showed a normal threshold and latencies on both sides. Otolaryngological and audiological examinations are important in the treatment and prognoses of children with this syndrome who have sensorineural or conductive hearing loss.