Study conducted at the Department of Speech-Language Pathology and Audiology, Universidade Federal de Ciências da Saúde de Porto Alegre – UFCSPA – Porto Alegre (RS), Brasil.
(1) Department of Speech-Language Pathology and Audiology, Universidade Federal de Ciências da Saúde de Porto Alegre – UFCSPA – Porto Alegre (RS), Brasil. (2) Escola Especial Para Surdos Frei Pacífico, Porto alegre (RS), Brasil.
Conflict of interests: No
Author’s contribution: MR principal investigator, development of research, development of the schedule, survey of literature, collection and analysis of data, article writing, article submission and procedures; DCGMV guiding, development of research, development of the schedule, data analysis, correction of article writing, approval of the final version; CRC co-advisor, collaboration in the development of the research and the schedule, analyze the data, correcting the article writing, approval of the final version.
Correspondence address: Milena Rizzon. R. Genésio Preto, 150, Desvio Rizzo, Caxias do Sul (RS), Brazil, CEP: 95110-320. E-mail: mi.rizzon@hotmail.com
Received: 4/17/2013; Accepted: 8/21/2013
Language evaluation in a case of association between
deafness and pachygyria
Avaliação de linguagem em um caso de associação entre surdez e
paquigiria
Milena Rizzon1, Deisi Cristina Gollo Marques Vidor1, Carina Rebello Cruz2
ABSTRACT
In order for the acquisition and development of language to occur, it is necessary to associate a number of factors, whose harmonious functio-ning determines the success of this process. The hearing loss may be an obstacle, but even so, the deaf child will be able to acquire language through a sign language. However, when the child shows neurological changes, the speech monitoring becomes necessary. This research is a case study of a deaf child, aged 5 years and 10 months, in the acquisition of sign language, who presents neurological impairment. It aims to as-sess, analyze, and deepen the knowledge on a rare disease (pachygyria) associated with deafness, showing the impact that this can pose to the child regarding communication. Data collection was done through formal evaluations and observational regarding the ability and modality used for communication, language development, phonological and lexical knowledge in Brazilian Sign Language (LIBRAS); in addition to a questionnaire. The results showed delay in language acquisition, even considering the gap between the child’s birth and the early acquisition of LIBRAS, aspects that may be related to the diagnosis of pachygyria. It is important that the audiologist know LIBRAS and know to assess the language of the child who uses this language, because cases like these reveal the complexity of the association between deafness and various neurological disorders.
Keywords: Case studies; Speech, Child Language; Lissencephaly;
Neurology; Deafness
RESUMO
Para que ocorra a aquisição e o desenvolvimento da linguagem é necessária a associação de uma série de fatores, cujo funcionamento harmonioso determina o sucesso desse processo. O comprometimento auditivo pode ser um obstáculo, mas, mesmo assim, a criança surda será capaz de adquirir linguagem por meio de uma língua de sinais. Porém, quando a criança apresenta alterações neurológicas, o acompanhamento fonoaudiológico se faz necessário. Esta pesquisa é um estudo de caso so-bre uma criança surda, com idade de 5 anos e 10 meses, em processo de aquisição da língua de sinais, com comprometimento neurológico. Tem como objetivo avaliar, analisar e aprofundar conhecimentos sobre uma doença rara (paquigiria), associada com surdez, mostrando os impactos que isso pode acarretar à criança, em relação à comunicação. A coleta de dados foi efetuada por meio de avaliações formais e observacionais sobre habilidade e modalidade de comunicação utilizada, desenvolvimento linguístico, fonologia e conhecimento lexical na Língua Brasileira de Sinais (LIBRAS), além da aplicação de um questionário. Os resulta-dos apontaram atraso no processo de aquisição de linguagem, mesmo considerando-se a defasagem entre o nascimento da criança e o início da aquisição da LIBRAS, aspectos que podem estar relacionados com o diagnóstico de paquigiria. É importante que o fonoaudiólogo conheça a LIBRAS e saiba avaliar a linguagem da criança utente dessa língua, pois casos como estes revelam a complexidade da associação entre surdez e quadros neurológicos variados.
Descritores: Estudos de Casos; Fonoaudiologia; Linguagem Infantil;
INTRODUCTION
The integrity and normal functioning of the hearing system, the maturation of the central nervous system, the appropriate cognitive development and the integrity of the phonoarticu-latory organs are necessary for the normal acquisition and development of the oral language(1). When any of these aspects
is compromised, communication disorders are inevitably obser-ved in greater or lesser degree, depending on both the affected aspect and the level of change.
The case results which will be presented in this article is related to an association between deafness and pachygyria, denoting change in the process of language acquisition, cau-sed by the impairment of the central nervous system, on the one hand, and the associated auditory sensory, on the other. Although pachygyria has no direct relationship with deafness, that may prove to be the cause of this, due to possible changes in the neurons of the central auditory pathway.
The pachygyria is a disease of the developing of the nervous system, caused by neuronal migration disorders. In this mal-formation, it is observed that the external aspect of the cerebral hemispheres has been changing, being the convolutions abnor-mally large, flat, reducing the number of grooves, resembling those of a fetus aged between 6 and 7 months. Can be found in all brain areas or areas located in one or in both hemispheres(2).
Closing the diagnosis of pachygyria is obtained by clinical evaluation and imaging tests. Neurological examination usually shows a delayed motor and language development, mental retardation, global hypotonia and balance deficits(3).
On the other hand, the auditory sensory impairment caused by deafness also generates deficits in the acquisition process of the oral language. The impact of this change depends on a number of factors such as the degree of loss, the time of de-tection of deafness and the use of tools that can assist the deaf individual(1). However, the lack of hearing, in isolation, is no
im-pediment to the organization of experienced reality through the language(4). The full control of this aspect of learning depends
on the functional components of linguistic competence - form (phonology, morphology and syntax), content (semantics) and intended use (pragmatics)(5). This competence can be acquired
either through the acquisition of a language of oral modality, like Brazilian Portuguese, as well as through the acquisition of a visuospatial modality language, like the Brazilian Sign Language (LIBRAS).
LIBRAS is officially conceptualized as a form of communi-cation and expression in the visual-motor system, with its own grammatical structure, it is a linguistic system of transmission of ideas and facts, just like any other language of the auditory--oral modality(6). It is understood that should become the mother
tongue or first language of the deaf population, when it is not possible to use the oral language for the communication(7).
When the deaf child receives stimulation from their mother tongue since birth, language delays are usually not checked,
as in hearing children exposed to spoken language. However, this is not the reality of most deaf children whose parents are hearing persons, unaware of LIBRAS. Failure to early exposure and continuing to this language, associated with lack of audi-tory feedback of oral languages, can compromise the child’s linguistic development(8).
Even considering the situation of optimal and early exposure to LIBRAS, deaf children also may develop changes in their process of language acquisition in sign language, and should be referred to speech therapy, with the aim of improving their lan-guage and improving their social interaction and quality of life. Furthermore, the presence of comorbidities that compromi-se other escompromi-sential aspects of the process of language acquisition, such as those affecting the cognitive and motor development, increases the risk of injury in this area(1).
Therefore, the importance of this study is justified by our aim to evaluate, analyze and deepen knowledge on a rare and complex disease associated with deafness, by showing the impact that this may have on the child in relation to their communication – mainly with regard to the process of langua-ge acquisition –, and by making a comparison based on the description of the literature on language acquisition of deaf children without comorbidities, which use LIBRAS as their first language.
CLINICAL CASE PRESENTATION
The present case study is of a female child, 5 years and 10 months old, diagnosed with pachygyria and sensorineural profound bilateral hearing loss. The pachygyria, with involve-ment of the frontal and parietal areas in the right hemisphere, was attested by a neurologist in 2011. The diagnosis of hearing loss was obtained in 2008 through behavioral, electroacoustic and electrophysiological evaluations. The guardian of the child signed the Informed Consent Form, agreeing with the realization and dissemination of this research and its results, in accordance with Resolution 196/96. This study was approved by the Ethics Committee of the Federal University of Health Sciences of Porto Alegre (UFCSPA) under the number 12/946.
Data collection was done through formal evaluations and observations of the child’s development in language mastery, involving aspects of comprehension and expression in the areas of pragmatic, skill and communication modality and lexicon and phonology in LIBRAS, along with a questionnaire applied to the responsible.
home, the child can communicate through LIBRAS, gestures and language itself. The family, listener, studied LIBRAS and knows some signs, but also uses gestures themselves to communicate to the child.
In order to assess aspects of language formally, three proto-cols were used: “Evaluation of sign language in deaf children in the school”(9), used to observe and record the child’s
develop-ment in language (comprehension and production), determining its acquisition level; “Protocol for Evaluating Communication Skills and Language for Deaf Children: “Pragmatic Profile of Deaf Children”(10), which assesses the pragmatic abilities and
communication mode of the child; “Proposal of an instrument for assessing phonological awareness, parameter configu-ration of hand for deaf children users of the Brazilian Sign Language”(11), which was used for lexical and phonological
proficiency assessment in LIBRAS. The evaluations were recorded by camera, to be analyzed at a later time.
“Evaluation of sign language in deaf children in the school” consists of tests for quantifying and monitoring the develop-ment (somatic and formative test, respectively). Was applied the formative test and part of production of the somatic test due to the child’s difficulties.
In the case of formative test, the professional has a tool for observing and recording children’s development in language, determining their level of acquisition. The criteria assessed refer to the understanding and expression of language, reference, content, cohesion, use and form. The development levels asses-sed are divided into 0 + (0 to 12 months) 1 (about 1 year), 2 (2 to 3 years), 3 (3 to 4 years), 4 (4 to 5 years) 5 (5 to 6 years): 6 (6 to 7 years) and 7 (11 to 13 years), according to the language characteristics of the child at the age.
To apply the test, you must determine the level of unders-tanding and use of language of the child through observation and playful activities; starting issues with the previous level; answering questions until you get a negative response; to de-termine the level at which the child is, which corresponds to the highest level, in which all the answers are marked positive; then you determine if the child shows the level (+), indicating that it has most of the questions given at a level immediately above the one in which it was classified.
The child in this case was initially classified at level 2 (2-3 years) according to their use and understanding of language. At level 2, it is expected that the child produce signals to talk about things and present actions, that the use of language is linked to the functions that seek to draw people’s attention, make requests and complains and communicates more than she is able to produce explicitly, enabling others to understand what she has left to say.
From this initial classification, the formal test was applied
and the girl remained at level 2, confirming the observation of the therapist (Chart 1).
To evaluate the production of this instrument, a story was selected – speechless cartoon “Tom and Jerry” (with seven events) – which the child should attend to and report to a third person who had not seen the cartoon. The evaluation of its production is descriptive, analyzing the use of mechanisms of the structure of sign language, such as setting of referents in the space, verb agreement and classifiers*.
The child watched the video for a minute and a half, paying attention all the time. When asked to tell the story, she used simple syntactic structure, using only notes and classifiers. First, she pointed to the computer, referring to the character and ran the classifier to increase the radio volume. Then she pointed again to the computer and mimicked the cat laughing.
The “Protocol Assessment of Communication Skills and Language for Deaf Children: “Pragmatic Profile of Deaf Children” is a list of communication skills, comprised of three sections: communicative intentions, response to communi-cation and interaction and conversation. These skills should be observed through a semi-structured playful activity. The analysis of the functions and communication skills should be made upon considering the presence or absence of each aspect and, in the case of presence, which mode of communication is used by the child - sign language, spoken language, gestures or vocalizations - and the linguistic stage in which she is - not language, oral language or linguistic signs.
The child performs most of the topics observed (92%) consistently. Some of the evaluated topics were conducted in more than one mode of communication, mostly in the gesture mode (76%), followed by sign language mode (60%) and vocalization (12%) (Chart 2).
The “Proposal of an instrument for assessing phonological awareness, parameter configuration of hand, for deaf children who are users of Brazilian Sign Language” is composed of two parts. However, due to age and some specific prerequisites, only the first part of the test was applied, which evaluated the lexical and phonological proficiency, that is, the knowledge of the informant regarding the requested vocabulary on the instrument and how these signals are produced.
In the instrument, the child should call 120 familiar pictures. Regarding the assignment of meaning and how the signals are produced, the achievements of the child can be classified into five categories: Expected Denomination (DE); Unexpected Denomination (DNE); Modified Expected Denomination (DEM); Comment, classifier or mime (C/CL/Mm) and Not denomination (ND). The DE occurs when the child makes the designation according to the picture and the target set and the DNE, when a name does not match the target set,
Chart 1. Questions marked in levels of “Evaluation of sign language in deaf children in school”
Level 1 (about 1 year)
Criterion Key Questions Yes Not
1 The child refers to the objects through the indication, holding, looking and touching them? X
2 The child communicates referring to toys, lights, objects, animals and food? X
3 The child begins to have the initiative and participates in other activities such as put and take objects with each other?
X
3 The child imitates movements produced by others? X
4 She uses a non-verbal language to draw attention to personal needs? X
4 She uses a non-verbal language to express their reactions? X
4 She uses a non-verbal language for help ranging your look between the object and the person who helps her pick up the object?
X
5 The child imitates signs despite showing hand configurations and movement imperfect? X
Level 2 (2 to 3 years)
Criterion Key Questions Yes Not
1 The child produces isolated words or signs to talk about things and actions around them? X
2 The child identifies the object to be referred to by another? X
3 The child sometimes she repeats what was said? X
4 The child uses language to make requests? X
4 The child uses the language to draw others' attention to her or her location? X
4 The child uses the language to say "hi", "bye", etc.? X
4 The child uses the language to complain? X
4 The child uses the language to name objects? X
4 The child uses the language to notice the presence of objects? X
4 The child uses the language to express that objects disappeared (or been removed)? (ended ...) X
5 Child points, looks, touches, identifies the things about which he is speaking with meaning? X
Level 3 (3 to 4 years)
Criterion Key Questions Yes Not
1 The child comes to the things of your interest? X
2 The child talks about things and events that involve? X
2 The child talks about the location of objects? X
2 The child speaks about the fate of the objects who withdrew or when they are being removed? X
2 The child talks about features temporary or about permanent of objects and things? X
2 The child speaks about the things that belong to her and the others? X
3 The child can relates what talks to what others are talking about in any way? X
4 The child uses the language to represent their actions? X
4 The child uses the language to notice the presence of several objects and to express that objects have disappeared (or been removed) and reappeared?
X
4 The child uses the language to make different types of requests? X
4 The child uses the language to identify things and actions into figures? X
5 The child uses consistently sentences with at least two components syntactically related? X
5 The child provides enough information for people to understand your intention? X
Note: 1 = reference; 2 = content; 3 = cohesion; 4 = use; 5 = form
but retains relationship to it and the picture shown. When the child performs the denomination according to the picture, but some parameter is modified during production compared to the standard signal (adult), it is considered a DEM. C/CL/Mm occurs when the child makes a comment on the picture such as “like mine” or a classifier, like manually to represent the shape
of the building of the picture presented, or even mime, to make the move with the foot ‘kicking a ball’. If the child does not call nor refers to know the signal corresponding to the picture displayed, this is labeled ND(11).
the test application, the examiner again requests the name of each picture whose production was not a DE. If the child produces, on reassessment, the fact is marked on the card in “comments”.
Out of the 120 assessed pictures, 17 (14.2%) were named as expected, seven (5.8%), as unexpected, seven (5.8%), as ex-pected from modified, 37 (30.8% ) were called with comment, classifier or mime and 52 (43.3%) were not referred (Chart 3). With respect to the signals produced by the child, the pho-nology was also evaluated. The question of phopho-nology in oral language refers to the study of sound systems. However, the phonology of sign language is established not from sounds as the minimal units, but from the form of the signals(11).
The hand configuration parameters (CM), pivot point (AP) and movement (M) are minimal units studied in phonology of sign languages, as well as the orientation of the hand (Or)
and non-manual aspects (NM), which refer to facial and body expressions(11).
The CMs are the various forms that the hand(s) take in the implementation of the signal. The M can involve a vast network of forms and directions, from the internal movements of the hand, of the wrist, to directional movements in the space, or to sets of movements in the same sign. The PA is the space in front of the body or a region thereof, where signs are articulated. The Or is the direction of the palm over the signs: up, down, forward, left or right. The NM refer to movements of the face, eye, head, or torso(11).
Out of the signs produced by the child, seven had DEM, or phonology was altered in some way, in any of the parameters. Of these, the CM was the parameter that had more change in the child’s productions, totaling four changes (57.1%), the PA was changed into two signals (28.6%) and the Or in one (14.3%).
In addition to formal evaluations, observations were made on all aspects of development, both in the therapeutic setting and in the classroom: The girl proves to be affective and quiet, but distracted. She does not maintain eye contact for a long time and the attention time directed to the signs and to the activities is abbreviated. She is only able to make signals and single orders with at most two components. The expressive language is even simpler, consisting of limited vocabulary. She performs only isolated signs — only some of which are understandable. She frequently makes imitation of signals, and performs symbolic behaviors to play. She presents broad and fine motor difficulties with noticed changes in gait and balance.
DISCUSSION
The discussion of this case, which associates pachygyria to deafness, compares the performance achieved by the rese-arch subject in evaluations of language with the literature data concerning expected standard development for deaf children, users of language of signals and born to hearing parents with varied linguistic exposure period (PEL). The results will also be compared to data from natural acquisition of LIBRAS, listing, whenever necessary, possible changes that may be attributed to the commitment generated by pachygyria. The main objective here is to verify the impact that this diagnosis is causing to the affected child, relative to her communication skills.
As for the assessment of the child’s development relative to language, performed by the first protocol applied, it is possible to identify a large gap, even if considering a delay in the start of the acquisition process. The protocol suggests that the child should be at level 5, being able to communicate in complex form from the standpoint grammatical and pragmatic and making himself understood by strangers’ people for her. If we consider the time of exposure to LIBRAS (language used for evaluation) one would think the level to be attained was 4, in which communication, though already broad and complex, is limited to specific and everyday situations. However, the lack
Chart 2. Topics marked on their related communication modalities, the “Protocol for the Evaluation of Communication Skills and Language for Deaf Children”
Topics Observation
Communicative intentions G V OL SL NS
Gaining attention X
Requesting X X
Rejecting X X
Greeting X
Expressing X X X
Naming X X
Commenting X X
Informing X
Answers for communication
Getting attention X X
Interest X X X
Understanding gestures X
Recognizing X
Understanding intentions X
Anticipation X
Responding with fun X X
Negotiation X
Interaction and conversation
Starting X
Keeping X X
Intelligibility X X
Presupposition X
Repair X X
Request clarity X X
Ending X X
Hearing X
Participating X
Chart 3. “Data Sheet - Part 1 of the “Proposed assessment tool phonological awareness, parameter configuration of hand for deaf children users of Brazilian Sign Language”
Nº Sinal Produção Observações
1 Yellow DEM PP modified
2 Rice CL With one hand mimicked the plate of rice, and with the other hand the rice, as depicted
in the figure
3 Airplane DE
4 Blue DE
5 Ballerina CL Imitated the dance movement that the dancer was doing in the picture, and showed that
the dancer was pink like her, using appointments
6 Balloon ND
7 Whale DU Produced the sign 'fish'
8 Cockroach DU Produced the sign 'bull' Expressed fear reaction
9 Tent DEM Denominated 'home' with modified PP
10 Baby DEM Produced the sign 'baby' with Or modified
11 Library ND
12 Bicycle Mm Held the pedaling motion with your feet, and hold the handlebars with your hands
13 Nozzle DEM HC modified
14 Bull DE
15 Ball DE
16 Cap DE
17 Rubber ND
18 Wild Mm Imitated the facial expression
19 Brazil ND
20 Witch ND Expressed fear reaction
21 Donkey DU Denominated 'bull'
22 Dog Mm Movement bite with your mouth
23 Chair C Pointed to the chair she was sitting
24 Bed CL She leaned back in her chair and represented manually format the bed
25 Kangaroo DE Once called 'kangaroo' made the sign 'bull'
26 Car DE
27 Home Mm Made to mimic the "knock" on the door of the house, in the figure
28 Slipper C She pointed to the picture and then to feet
29 Chocolate Mm Gesture of "bite the chocolate"
30 Rain DE
31 Five DEM HC modified
32 Gray ND
33 Snake Mm Produced bite with her mouth, and movement of the snake boat. Expressed fear reaction
34 Coconut Mm Produced movement to break the coconut and tapped the table, expressing "hard"
35 Rabbit DU Produced the sign 'bull'
36 Computer Mm Made the gesture of typing something on the computer keyboard, in the image itself
37 Glass C Referred 'drink'
38 Given ND
39 Sweet Mm Made to mimic "eat the candy"
40 Two ND
41 Building ND
42 Elephant DEM HC modified
43 Toothbrush DE
Nº Sinal Produção Observações
45 Mirror CL Mimics the girl looking in the mirror. Repeat the signal of the examiner immediately
after viewing the same
46 Knife ND Does repetition
47 Bean Mm Made to mimic "eat the beans" Tries to repeat, but uses HC and M not similar to those used in the production of the standard signal
48 Son C Points for her and the computer
49 Soccer Mm Produces mime kicking the ball and the signal 'ball'
50 Gelatin ND Tries to repeat, but uses HCs not similar to those used in the production of the standard signal
51 Giraffe CL Makes the ears of the giraffe. Makes imitation
52 Umbrella DE
53 Helicopter DU Denominated 'airplane' and did mime "piloting"
54 Man C Commented on the man's hair
55 Alligator ND Demonstrated fear
56 Newspaper ND
57 Lion DE
58 Milk CL Classifier used, "serving milk"
59 Lemon CL Produced the form of lemon
60 Book ND
61 Wolf DU Denominated 'Lion'
62 Moon C Refers 'bedtime'
63 Light on ND Makes imitation
64 Light off ND
65 Apple Mm Made the movement to "eat the apple"
66 Monkey C Commented about the teeth of the monkey
67 Mother C Pointed for her and for the figure
68 Socks C Shows their socks
69 Watermelon Mm Movement "bite" the watermelon
70 Table C Pointed to the table which we were using
71 Corn ND
72 Backpack DEM HC modified
73 Bat CL Makes the movement of the wings of the bat. Tries to make imitation
74 Mosquito ND Uses facial expression of fear
75 Motorcycle DE
76 Woman ND
77 Boat DE
78 Nine ND
79 Clouds C Commented "it rains"
80 Eight ND
81 Ounce Mm Did the movement of bite
82 Bus DU Denominated 'car'
83 Eleven ND
84 Father C Pointed for her and for the figure
85 Clown CL Mimicked the clown. Does repetition
86 Bread ND
87 Santa Claus ND
Nº Sinal Produção Observações
88 Stone ND
89 Popsicle DE
90 Kite ND
91 Pig ND
92 Present ND
93 Black ND
94 Cheese ND
95 Mouse ND
96 Soda Mm Did a gesture of "open" the soda
97 King ND
98 Laugh ND
99 Robot DE
100 Rose ND
101 Skirt ND Searched for someone wearing a skirt
102 Shoe ND
103 Seven ND
104 Sun ND
105 Soldier C Referred 'run' and 'shoot'
106 Phone ND
107 TV ND
108 Tennis ND
109 Towel ND
110 Train ND
111 Sad ND
112 Toucan ND
113 Bear ND
114 Grape ND
115 Broom DE
116 Candle ND
117 Green ND
118 Dress ND
119 Videocassette ND
120 Cup and Saucer ND
Note: DE = denomination expected; DU = denomination unexpected; DEM = denomination expected modified; C/CL/Mm = comment, classifier or mime; ND = not denominated; HC = hand configuration; M = movement; PP = pivot point; Or = orientation of hands
Quadro 3. continuation
of components that may make the child’s communication more understandable and complete is observed. Even with this delay, it can make use of language to achieve their goals, referring to the things you need with isolated signs and gestures and comprising phrases with up to two elements.
The assessment carried out by the same instrument, in re-lation to signs linguistic production, also shows a gap between the expected and the achived. According to the pilot implemen-tation of this protocol(9), all students surveyed used classifiers
satisfactorily during production, which did not occur with the child of the present study, which used this classifier – considered
the most basic – but which was scarcely accomplished through simples syntactic structures. A verbal agreement, even though the most complex aspect of the production(9), should be used
more fully at that age. However, the child has not reached the expected performance because the story had not a context nor continuity, as it should be reported by two sentences with binding.
of visuospatial modality (sign language), followed by oral language (LO) and the use of gestures and vocalizations, sho-wing greater linguistic complexity to sign language. The child in this study has a lower mastery than expected for her age and experience with LIBRAS as well as she has not used all communication skills appropriately; upon doing so, she mostly did it through gestures as a way to communicate because of the difficulty in the acquisition of sign language.
It is important to note that, despite this, the child performed most of communicative skills elicited by the test. The literature reports that these interactions stimulate the communication and intellectual development of the child and can interfere with the speed of its development and the linguistic field(5). Interesting
to note, also, is that the two skills are not performed by the child - “listening” and “participating” – are conversational interactions with two other people. This datum corroborates with the literature(13), which shows that hearing children with
a cognitive deficit have difficulties in social interactions with other people.
Thus, it was established that the child is in linguistic stage which is the stage of a sign, visible delay relative to language. Comparing with the language acquisition of deaf children, children of deaf parents, exposed to native language from birth, a study showed that the early stage of a signal is at around 6 months old, going up to 2 years(11).
Regarding to the lexicon, considering the results of the third evaluation instrument applied, the 15 children participating in the study were able to name all 120 pictures of the instrument. The children presented DE, DNE, DEM, C/CL/Mm, as well as the subject of this research. However, the data(11) show that
the proper level of recognition of pictures (DE) was 77.4% for the 15 children, while the subject of this research named only 14.2% of the pictures as expected**.
This result points to a restricted vocabulary for her age. However, as it was also examined in other instruments, the attempt of the child to signal when she wanted to show that she knows something, was clearly. She showed that she recognizes more than half of the figures (56.6%), because she could name them or attempted to, somehow.
In this same study(11), it was also possible to verify that,
according to the increase of PEL, there is a tendency for more expected names to occur. Children under 4 and a half years of language exposure, such as the one in this study had a higher incidence of DNE, C/CL/Mm and ND than those with more than 6 and a half years of exposure. However, the most frequent (62.5%) of this group was still of expected names. Nevertheless, not even regarding the PEL the child in this study reached similar average in vocabulary when com-pared to children without comorbidities. Therefore, the time of deprivation of language, even collaborating for the girl’s
language delay, suggests that this is not the main triggering factor of this fact.
The phonology was also evaluated by this protocol. According to the literature(14), relative to the order of
acquisi-tion of phonology in sign language, the PA is the first aspect to be acquired, followed by M and CM. We observe also that the substitutions of these aspects become less frequent at older ages. Of those parameters, the CM was the most changed by the child and the PA, the least. Taking into account that the girl is at the stage of language acquisition, it is acquiring the parameters in the sequence provided, and even more difficulty than other children, it is expected that the substitutions that she performs become less frequent with the increase of age and the PEL.
The signals produced by the child are mostly with a CM, with one hand (1m 1cm) or both hands (2m 1cm). There was a single output signal, expected form with both hands and CM different between them (2cm 2m) - “varnish”. Even in situations of imitation, it was veryfied that the child is unable to produce signals according to the standard adult ones, with 2m 2cm - “Bean”. According to the data found in the validation study of this protocol(11), the signs formed by a CM have more DE than
signals formed by 2m 2cm.
Some authors offer explanations for this fact. One is that the child must understand the correct form of the CM in both hands. A second explanation is that some CM exhibit a relati-vely small area as a point of contact, requiring a degree of fine motor control unusual in younger children. Finally, forming signs with both hands and distinct CM requires a relatively advanced level of coordination(14). These explanations are
consistent with those data provided by the child of this study, who has faced significant motor difficulties, due to her diag-nosis of pachygyria.
According to the literature(11), children at the age and PEL
similar to the subject child, must already have acquired more signs with 2m2cm. The expected index name was 68.33% for these signs, while the girl in this case study named only 6.25%, as expected.
Since the vocabulary of the native language provides inte-gration with the social environment, the semantic implemen-tation will be possible as the phonological inventory increase with the number of phonemes. Thus, the development of the lexicon and phonology is connected, even if there are indivi-dual variations(11). In this case study, the vocabulary may be
compromising other important areas for the acquisition and appropriate development of language.
Researches that assessed the language of deaf children with some neurological impairment associated were not found in the literature. However, consideration of cases will be brought about listeners, since the acquisition of language by deaf children can occur in the same period and with the
same stages observed in hearing children, considering some conditions regarding the linguistic input that deaf children have access(11).
Hearing children who have a cognitive deficit have diffi-culties in language acquisition and literacy, presenting more receptive language preserved than the expressive, and this is little elaborate, consisting of short sentences with few con-necting factors, very often restricted to the verb and the direct object, simple vocabulary, with little creativity in the narrations of stories. She also presents global neurodevelopmental delay, severe impairment of attention, of memory, of visuospatial ability and of fine motor control, as well as of social skills. She shows little interest in therapy and pedagogical difficul-ties, and her evolutionary rhythm of cognitive capacities is extremely slow. Most children’s first words began late, with even difficulty forming sentences and reading. Patients with mental retardation had higher cognitive deficits in vocabulary test, although it is observed communicative intention, despite the linguistic difficulties(13).
All the difficulties described above confirm the diagnosis of the child who is subject of this research. Thus, deafness and early exposure to LIBRAS, from 1 and a half year age, are not, by themselves, causing the delay in language acquisition of child. Other extralinguistic factors, but are strongly linked with language, caused by cognitive deficits, such as impairment of attention, memory, and visuospatial and motor aspects, affect the acquisition of LIBRAS, especially for being a language of visuospatial modality and require fine motor movements for signaling.
Even if we consider that the child has a diagnosis of pa-chygyria in the right hemisphere, we see that this hemisphere is associated with the cognitive functions of attention, visuospatial perception and memory, body image, social and emotional intelligence and recognition of facial expressions(15), functions closely linked to aspects necessary for the use of LIBRAS.
The literature shows that there is an association between the delay in language development and the total number of communicative acts of deaf children, i.e, those that interact more, have less delay in language development(5). Although
all data of evaluations have been shown lagged, the girl of this study revealed great communicative intention, despite her limitations, and this may be helping her to achieve an effective communication.
Children with cognitive deficits who have delayed motor and language develop in the future, present several academic difficulties in various domains of cognition. This reflects the need to develop strategies for early pedagogical and speech intervention for the group of children with this diagnosis, because the academic difficulties are already provided. After this early stage, these children eventually adapt to restricted social environment and do not follow the school activities, as reported in a study with adolescents, whose advances were very limited after this period(13).
The findings of this study have important implications for language intervention programs with deaf children. In addition to promoting a deeper understanding about the pachygyria, which can also be extended to other cognitive deficits, whi-ch, when associated with deafness, should receive special attention from health professionals, this study may facilitate the treatment planning by these professionals, and provide for them a broader point of view in cases of patients with cognitive deficits who use other mode of communication, so that they have all possible assistance and a competent inter-disciplinary team involved and working in their favor. Finally, we believe that this study will contribute to the analysis of linguistic evolution in the speech-language therapy process in communicative context.
FINAL COMMENTS
The study participant showed significant delay in language understanding, but mainly in expressive language, with great lag of vocabulary. However, she demonstrated pragmatic abi-lities and great communicative intent. An important finding was that the time of linguistic deprivation suggests not be the main trigger factor for the delay in the language acquisition of children, being the pachygyria that is intervening more evidently in this process.
It was perceptible during the application of the proposed tests, that the lack of attention and visual contact were really hindering the child in her communication, they are essential for the development of any language, but especially for the sign language. The communicative intention, which was also perceived throughout the evaluations showed that the girl does not cease to communicate – although she makes preferential use of ungrammatical means, in a linguistic point of view – which has been essential to her development.
Children with difficulties in the area of language, even when they are users of LIBRAS, should receive all the support and stimulation of people living with them directly. In this case, the use of LIBRAS by the child’s family is extremely important, as well as by teachers and fellow students, and by the professionals from the clinical area, so that they may have more chances to develop linguistically-wise, more quickly and more appropriately.
In this approach, an audiologist fluent in LIBRAS may contribute to the identification of priorities and communica-tion needs of the child, setting goals and providing accommunica-tions that contribute for the reduction or for the overcoming of the
communication lags. Thus, signs language children can receive
support and stimuli necessary for their better development, providing them with better quality of life.
ACKNOWLEDGMENTS
The “Universidade Federal de Ciências da Saúde de Porto Alegre (UFCSPA)”, by incentive to research, case number 12/946.
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