Table IV.

Perception scores and expressive language outcomes in children with CHARGE syndrome.

Patient	Perceptive category				Language development
	Pre CI/HA	After 6 months	After 1 year	After 3 years or more	Pre CI/HA	After 6 months	After 1 year	After 3 years or more	Italian sign language development
1	1	3	5	5	1	2	3	3	+
2	N/A				N/A
3	0	1	3	3	1	2	2	3	+
4	3	3	3	3	2	2	2	2	+
5	0	1	1	1	1	1	1	1
6	0	1	3	3	N/A	1	2	2	+
7	0	1			1	2
8	0	1	4	3	4	4	4	4
9	2				2
10	N/A				N/A
11	6				6
12	0	1			1	2			+
13	0	0			2	2			+
14	4	4	4	4	2	2	2	2
15	N/A				N/A
16	0	2	3	4	1	2	3	4	+
17	1	2	2		1	2	2		+
18	NO HA 5				6
19	1	1	2	2	3	3	3	3	+
20	3	4	5	5	3	3	5	6
21	0	1			1	1
22	0	0 CAND CI			1	2			+
23	3	4	4		1	2	2
24	0	0	0		1	1	1
25	0	1	1		1	2	2		+
26	0	1	1		1	2	2
27	NO HA 2				1
28	0	1			1	1	1
29	0	1	2	4	1	2	3	3	+
30	0	0 CAND CI
31	4	5	6	6	5	5	5	6

AUDITORY PERCEPTIVE PERFORMANCE (Geers, Moog, 1987 mod. ²⁸)

0 = no detection of speech sounds

1 = simple detection

2 = pattern perception

3 = inconsistent closed-set word recognition

4 = consistent closed set word recognition

5 = open set word recognition

6 = open set word recognition (exceeding performance with old device)

MAJOR STAGES OF LANGUAGE DEVELOPMENT (Bates, O'Connel, Shore, 1987 mod. ²⁹).

1 = absent = voicing/babbling

2 = voc =vocalisations/CVC sequences to communicate intentionally

3 = words = first words/single word utterances that have communicative contents

4 = combinations = first words combinations/telegraphic utterances

5 = sentence grammar = combinations based on morphological and syntactic rules

6 = discourse grammar = connected discourse closely conformed to adult model