Hipoacusia neurosensorial en un síndrome de Noonan y secuencia Poland

it is estimated that the 50% of cases of deep deafness during childhood may be or genetic origin. this is the case of a child aged 9 seen in otorhinolaryngology and genetics services of the "wiliam soler" teaching children hospital due to a unilateral severe neurosensory hypoacusis and mondini's congenital dysplasia in left ear contralateral to the major pectoral muscle hypoplasia, an interesting situation. hypoacusis was confirmed using tone audiometry and auditory evoked potential of brain stem. ear tomography demonstrated a cochlear hypoplasia with agenesis of apical spiral. the clinical manifestations and the significance of the ontological and imaging study in diagnosis of auditory loss are emphasized.