Abstract
Clinical observations of two girls with Landau-Kleffner syndrome were reported. The patients developed this syndrome at ages 4 (case 1) and 5 years 8 months (case 2), respectively. Auditory language disorders preceded, expressive language also deteriorated gradually, and abnormal EEGs were indicated. No other neurological abnormalities were found. Language development had been normal until the onset of the auditory language disorders.
In case 1 hearing sensitivity showed a normal threshold by pure tone audiometry and ABR. Nonverbal recognition elicited all correct responses. However, speech discrimination was very poor. Although 14 years had passed from the onset of these disorder, speech discrimination had demonstrated almost no recovery. Nonetheless, abnormal EEG disappeared by the age of 12.
In case 2 pure tone audiometry testing and ABR showed almost normal results. On the other hand, speech discrimination was very poor as in case 1. The non-verbal recognition score was 70 %, unlike case 1. Spontaneous speech gradually became vague. However, after several months from the first visit to our clinic speech discrimination began to recover. After receptive and expressive language disorders disappeared, abnormal EEG continued.
This paper traces these two cases and discusses clinical features of cases with Landau-Kleffner syndrome, subdivided into those characterized by pure word deafness (case 1), auditory agnosia (case 2), and aphasia.
