Although newborn screening of congenital hearing loss through otoacustic emissions allow prompt recognition, imaging techniques, such as CT and MRI are needed to get a morphological diagnosis. Furthermore they can be very useful in unilateral cases, whose clinical presentation is belated and more insidious. Our aim is to show the utility of MRI in the study of inner ear congenital anomalies, whose presentation is belated. Thus from a series of 88 consecutive patients in which a MRI was performed as screening of assymetric sensorineural hearing loss, we selected 6 cases aged between 6 and 20. Four of them showed an inner ear anomaly on MRI. We present these anomalies commenting the findings on CT and MRI. Imaging techniques are required to start hearing rehabilitation programs early on patients with bilateral inner ear anomalies. But also they are very useful in the evaluation of unilateral assymetric sensorineural hearing loss, in young patients, even if only some frequencies are damned, to determine the nature of hearing loss.
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