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Audiogram of family members tested. Thresholds for audiogram against the tested frequency plotted in comparison with age-based standardized data. Data of the subjects II-4, II-3 and II-2 are compared with standardized age-based data (
age-group 30–39), data of subject II-1 are compared with age-based standardized data (
age-group 40–49) and the data of subjects III-3 and III-4 are compared with age-based standardized data (age-group 20–29
) recorded by Bungert-Kahl et al
. (2004) respectively. Gray box plots (
) show the 5% and 95% percentile and dark gray line and scatter plots (
) show the median and 95% confidence interval of the respective threshold for all recent tested normal hearing subjects of corresponding age group (II-2 was not able to hear test tones with a frequency of 16 000 Hz).
Figure S2: (a) Individual discrimination threshold values as z-scores referring to age-matched normative data (ordinate, logarithmic scale) for intensity (top panel), duration (middle panel) and frequency (lower panel) discrimination tests under monaural (left column) and dichotic (right column) stimulus conditions plotted separately for all family members (abscissa; arranged by increasing age). Dashed horizontal lines at 1.6 indicate the significance limit. Note that z-scores above 1.6 suggest impaired discrimination ability compared to age-matched normative data. All affected family members are displayed in color: II-3, II-2, III-3, III-4, III-5 and III-6. Open symbols for III-5 and III-6 indicate data from a separate second session conducted 4 years after the first session. (b) Cumulative distribution of discrimination threshold values (z-scores referring to age-matched normative data) of family members diagnosed with central auditory processing disorder for different discrimination tests (discrimination of frequency, intensity, and duration differences) under monaural and dichotic stimulus conditions compared to the respective values of one exemplary control group (15-year-old subjects). The values for the different discrimination tests are sorted and ordered by size (abscissa) between 0% and 100% (ordinate). Median, 10% and 90% percentiles are presented by dotted lines, and the z-score at significance threshold is indicated by the dashed vertical line at +1.6. Note that 35% and 53% of z-scores for duration and frequency discrimination, respectively, exceed the significance level.
Figure S3: Acoustic parameters of stimuli used in the ERP experiment. (a) Lengthened syllable /ba:/. (b) Short naturally produced syllable /ba/. (c) Represents the first 30 milliseconds of (a) and (b) which were identical for both stimuli.
Table S1: Psychoacoustic test parameters for audiogram and discrimination tests: F = frequency, Hz = Hertz, I = intensity, dB = decibel, T = time, ms = milliseconds.
Table S2: Performance in central auditory discrimination test for duration, frequency and intensity for mono = monoaural and dicho = dichotic presentation.
Primer sequences used in candidate gene sequencing. Reverse primers are shown in the reverse complimentary direction. If an exon was too long to sequence from one product it was divided into two or more, and the products given the title ‘a’, ‘b’, ‘c’ etc. The different splice forms of CNTN1
were identified from UCSC genome browser build 36.1 and use the annotation from this website (http://genome.ucsc.edu/
Table S4: Relation between categorization of normal or affected/borderline in the NWR and the ERP pattern of each subject of the NE family. For classification criteria for NWR performance see and for classification of ERP pattern see . Note that the present analysis only considers the absence of MMN and enhanced LDN as indicating affected (code: I-III indicates the three generations, roman numbers indicate different family members in one generation) (see also ).
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