Aphasia and congenital deafness: A case study

Aphasia and congenital deafness: A case study

BRAIN AND LANGUAGE Aphasia 12, 28.5-291 (1981) and Congenital Deafness: A Case Study JUDY K. UNDERWOOD University of Northern Colorado AND ...

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BRAIN

AND

LANGUAGE

Aphasia

12, 28.5-291 (1981)

and Congenital

Deafness:

A Case Study

JUDY K. UNDERWOOD University

of Northern

Colorado

AND CONNIE Arapahoe

County

J. PAULSON School

District

No.

6

The case of a 57-year-old congenitally deaf man who suffered a cerebral vascular accident is presented. Resultant symptomatology included right hemiplegia and aphasia. The authors’ findings and conclusions concur with a small, but slowly growing, body of literature which suggests that the fundamental linguistic processes are the same for the congenitally deaf and for the normal hearing population.

It is difficult to make generalizations about aphasia among congenitally deaf adults since there is such a dearth of information in this area. At the time of this investigation, the few articles in the literature were limited to case studies, with most of the conclusions based on inferences made from a single case and applied to the general deaf population. Underwood (1972) explained this lack of research by: (a) the problem of finding an adequate number of subjects, and (b) the problem of communication between hearing people and congenitally deaf people. Aphasia is a relatively rare disorder in the general population, and is certainly no more common in the congenitally deaf population. Consequently, it is unlikely that there would be many congenitally deaf aphasics in any one location at the same time. The majority of hearing people are unable to communicate adequately with the deaf. Additionally, many speech-language pathologists who are trained to help people with aphasia do not know sign language, and may even be prejudiced against its use. The use of the term “deaf-mute” by Hughlings-Jackson (1878) and Grasset (1896) is obviously dated; however, their conclusions postulated in the 19th century seem to be in agreement with what authors have reported in the past few decades. Jackson wrote, “No doubt by disease of 285 0093-934X/81/020285-07$02.00/0 Copyright @ 1981 by Academic Press, Inc. All rights of reproduction in any form reserved.

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some part of his brain, the deaf-mute might lose his natural system of signs” (Hughlings-Jackson in Sarno, Swisher, & Samo, 1969). Grasset (1896) published a report of his patient who was “an intelligent deaf man” who had never learned to speak. The paralysis of his right arm was not severe enough to explain his impairment in sign language, and Grasset concluded that, “he really is, therefore, genuinely aphasic in the right hand, in the true and only meaning of the word.” Sarno et al. (1969) generalized their findings concluding that, “aphasia in the congenitally deaf is entirely equivalent to that in normal hearing people.” Tureen, Smolik, and Tritt (1951) stated that “the apraxia for finger speech is as much related to lesions affecting Broca’s convolution and its association pathways as is the apraxia for articulative speech.” Douglass and Richardson (1959) supported the concept of a “language zone” in the dominant hemisphere of the congenitally deaf. An additional description of this rare phenomenon is reported below. CASE REPORT Background

Data

Most personal information was provided by the patient’s wife, who was also deaf. Although she had adequate speech skills, she stated that she communicated to her husband entirely by manual communication. She reported that at 9 months of age her husband had a right mastoidectomy, and at the age of 12, a tumor was surgically removed from the left ear. She reported that although an audiogram was not available, she had never known her husband to respond to any auditory stimuli. Congenital deafness was documented in the medical report, and amplification had never been utilized successfully. At the age of 7, Mr. W. was enrolled in a state school for the deaf and blind, where nearly all communication with the students was manual. Following the second surgery, at age 12, he returned to his home on the farm where he lived and worked without any additional formal education. He seldom communicated with his family, since they were not fluent in sign language. At the time of his stroke, this 57-year-old man was employed as a factory worker, where he worked the night shift. He was married and had two grown children with normal hearing. Signing was the main means of communication used in interactions in the family. Mrs. W. reported that prior to the stroke, Mr. W. signed effectively, having learned manual communication from his classes at the school for the deaf and from his interactions with his deaf peers. In addition to American Sign Language, gestures were incorporated into his communication with deaf peers and he used signing more than fingerspelling. Mr. W. excelled in math, and often utilized mathematical concepts in his work. His writing skills were functional for basic needs, such as writing a check and signing his name. Minimal speech reading skills were observed. Apparently, the only time Mr. W. used speech was when

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speaking to his dog and when cursing. Vocal quality at these times was reportedly characterized by an elevated pitch, tense-strident quality, and breathiness. Basically, his speech was unintelligible. Neurological Information The neurological report indicated a left middle cerebral artery thrombosis with questionable ischemic infarct. Right flaccid hemiplegia was noted. Since this patient had always been left-handed, he was signing with his nonparalyzed extremity. It was hypothesized that the dominant hemisphere for speech and language must have been the left hemisphere, due to the resultant aphasia. Speech and Language

Evaluation

Portions of the Porch Index of Communicative Abilities, and the Minnesota Test of Differential Diagnosis of Aphasia were administered, although their statistical validity might be questioned due to the necessary alterations required because of the existing deafness. The patient was not required to produce speech, and all subtests were signed by the examiner. An audiological evaluation, including air and bone conduction was also administered. There were no responses to any frequency level. Observations

and Sample Beha\!iors

Communication skills appeared to approximate that of oral aphasia and apraxia, although the responses were elicited manually rather than orally. Mr. W. could inconsistently sign the names of simple objects; however, word-finding deficits, word substitutions, jargon fingerspelling, and perseverations were noted. For example, Mr. W. would often shake his head “no,” indicating his inability to complete the task when asked to sign or fingerspell the name of an object. He substituted the fingerspelling of “shoe” for “pencil.” He fingerspelled “Konil” when asked to sign “shoe.” After he named one item appropriately, he would often continue to name subsequent items by repeating the fingerspelling of the initial word. It was often impossible to reelicit responses which were formerly correct. Occasionally, he could fingerspell a word, sign the word, and yet not point to a picture of the word. Although before his stroke he was signing more than fingerspelling, following the stroke he was more often accurate when using fingerspelling. This might be attributed to the fact that fingerspelling only required one hand; whereas, when both hands were needed to sign a word, the task became more complex. He was not able to express his basic needs, even to his wife. Eventually, they devised a system of gesturing and pointing to make a few basic needs known, but Mr. W. never regained functional communication skills. Overall performance seemed best on an imitative level, although occasionally he could imitate a signed or fingerspelled response, but showed no apparent com-

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prehension of the concept signed. When incorrect responses were elicited, he seemed to be aware of his incorrect production, realized the receiver could not comprehend what he had signed, could not selfcorrect, and became very frustrated. In fingerspelling, occasional letter substitutions were made which approximated the intended letter. For example, he confused /s/, /a/, and /e/, all of which have similar hand positions. Occasional “limb apraxia” was noted. Searching, posturing behavior was observed when he was unsuccessfully attempting to fingerspell a word. When given the initial letter of a word, he readily completed the fingerspelling of the word, but did not appear to comprehend the name of the object he had just fingerspelled. Some automatic signing was noted. He could sign from I to 10 as well as sign the Lord’s Prayer. In one instance, he even corrected the speech-language pathologist’s signing of the Prayer. An inconsistent yes/no response was noted to simple questions, and usually his signed response was more reliable than his head nod. Receptive difficulties were noted in comprehension of basic signs. It appeared that he comprehended novice signers better than fluent signers, probably because they went slower, were more repetitive, and used easier signs. At times he could sign appropriate responses to simple oral questions from the clinician; for example, “What is your name?” “Who is your wife?” Mr. W. was unable to read simple words. He could not match the object or a picture of the object with the written form. Mathematical skills, which were a strength prior to the CVA, were functional only on a simple addition-subtraction level. Occasionally he could correct an error if directed to recheck a specific mathematical problem. Jargon was noted in graphic performance, as well as signed responses. For example, he wrote “raidors” for “cigarettes.” Word substitutions were also noted (“fish” for “toothbrush”). He could write his name, age, and wife’s name, but in response to “Where do you live?” wrote “I took her for more books.” Performance appeared to be best on simple written tasks. Mr. W. was highly frustrated and was obviously aware of his inability to communicate. His performance on all tasks was inconsistent. Course of Treatment Three weeks following a cerebral vascular accident, Mr. W. was seen at a rehabilitation hospital for intensive speech-language therapy, physical therapy, and occupational therapy for a period of 24 months. Each service was provided on a twice daily basis, 5 days a week. At the time of his discharge, Mr. W. was ambulatory when using a quadcane. Following his discharge to his home, outpatient therapy was scheduled three times a week. Six months after his inpatient discharge, he fell while walking from his living room to his bathroom. After this fall, he refused all treatment. Approximately 6 months later, he expired, reportedly from another cere-

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bra1 vascular accident. No autopsy was performed wife denied permission. Improvements

Noted

Since Initial

289

at his death since his

Referral

Although objective improvement was not seen in communication skills, some accomplishments seemed noteworthy. Mr. W.‘s family accepted the communication problems, and attempted to assist him in making his basic needs known. Prior to his fall, Mr. W. appeared to cope with his condition with decreasing frustration over time. His family devised a means of gesturing and pointing so that he did communicate a few basic needs. He “read” the daily newspaper. Although the actual comprehension was questionable, it was obvious that he derived pleasure from the activity. Slight improvement was noted in the reliability of his yes/no responses. Throughout therapy he gradually improved in his ability to comprehend simple fingerspelling and signing. DISCUSSION

Our findings concur with all previous reports, indicating that the resultant behaviors following a cerebral vascular accident are not different in congenitally deaf and hearing patients. Although communication modalities differ, the data consistently lead one to believe that the pathology is the same. There is some disagreement in the literature about the different effects of aphasia on fingerspelling and sign language, and no consistent pattern has emerged showing one to improve before the other. Those readers unfamiliar with manual communication should note that most congenitally deaf people communicate with a combination of signs and fingerspelling. American Sign Language (ASL) is concept oriented and uses syntax and grammar which are different from those of the English language. Words that have no signs are fingerspelled. Fingerspelling enables one to spell with the fingers and is analogous to writing in the air. In reviewing five individual cases, two did better in fingerspelling and the other three did better in sign language. Sarno et al. (1969) presented a case study of a 69-year-old congenitally deaf, aphasic man. Premorbidly, he communicated primarily through sign language. The authors were unable to determine how he communicated in his preschool years, and did not know whether he learned fingerspelling or sign language first. He showed greater improvement in using signs than in fingerspelling. The case report of a 21-year-old congenitally deaf woman by Douglass and Richardson (1959) revealed the opposite pattern (i.e., greater improvement in fingerspelling than in sign language). The patient in the present study has also revealed this latter pattern of recovery; whereas, the patient studied by Tureen et al. (195 I) reportedly recovered in sign language long before he could fingerspell. Battison (1976) found

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that his patient showed most of the errors in signed English and fingerspelling productions rather than in American Sign Language. Such differences are understandable in light of the many different variables influencing recovery. For example, in the presence of hemiplegia, signing ability might be more severely impaired than fingerspelling when both hands are needed to complete a sign; however, when only one hand is used for signing, fingerspelling might be more severely impaired. Sarno et al. (1969) suggested that there is not a hierarchy of difficulty among different methods of communication employed by congenitally deaf people who have aphasia. The authors indicated that the pattern of recovery will be dependent upon the extent and site of the neurological impairment. One might hypothesize that since the most recently acquired functions are the last to be recovered in aphasic patients, fingerspelling would be more severely impaired. It is possible, however, that some deaf students may have learned fingerspelling before sign language. Although it is true that children may have learned the alphabet through fingerspelling very early in the educational curriculum, it is probable that most of the children would either have already learned sign language before coming to school (e.g., if there were older deaf members in the family), or they would have learned sign language and fingerspelling simultaneously. This seems logical since sign language is an easier, quicker, more effective means of communication than fingerspelling. Aphasic characteristics in signing seem to be equivalent to those in speech; whereas aphasic characteristics in fingerspelling seem analogous to those in writing. The fact that deaf people’s language skills and communication habits are dependent on several variables confounds the problem. In one case (Battison, 1976), the patient did not acquire his hearing loss until the age of 5, which certainly would be a contributing factor influencing the patient’s command of English. Battison (1976) noted the difficulty in obtaining linguistic information about deaf aphasic patients. Unlike hearing aphasic patients, where a premorbid baseline of communication skills can be assumed, deaf aphasic patients’ premorbid communication skills vary significantly. An evaluator rarely obtains accurate information about a deaf aphasic patient’s prior linguistic skills. Consequently, in such cases it is difficult to determine the degree or quality of impairment that is directly related to the aphasia rather than the hearing loss. Douglass and Richardson (1959) found symptoms in their congenitally deaf aphasic patient similar to those seen in the hearing aphasic population. This client perseverated frequently and often abbreviated words and sentences. It was noted that many of her errors in fingerspelling were substitutions of letters which were closely related in position to the correct letter (e.g., d/f). The authors compared these incorrect responses to typical expressive aphasic errors in vocal expression of substituting a

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similarly articulated sound for the desired sound. Battison (1976) also found many similarities between aphasia in the hearing population and in a 75year-old deaf male executive, who suffered a CVA. A premorbid writing sample indicated this patient had an excellent command of written English; therefore, the researcher was able to conclude that many of the language problems found after the CVA were characteristic of aphasia, and not related to the patient’s deafness. Perhaps as Markowicz (1973) suggested, the type of aphasic patient who could provide the most valuable data on this subject would be someone who was truly bilingual in sign language and oral communication skills, for example a hearing person whose parents were deaf. At this time, no such case has been reported. REFERENCES Alajouanine, T., & Lhermitte, F. 1964. Non verbal communication in aphasia. In A. V. S. de Reuk & M. O’Connor (Eds.), Symposium on disorders of Innguage. Boston: Little, Brown. Battison, R. 1976. Linguistic aspects of aphasia in a deaf signer. Unpublished manuscript, Northeastern University. Critchley, M. 1938. Aphasia in a partial deaf-mute. Bruin, 61, 163-169. Douglass, E., & Richardson, J. C. 1959. Aphasia in a congenital deaf-mute. Bmin, 83, 68-80. @asset, J. 1896. Aphasie de la main droite chez un sourd-muet. Le Progres Medical, 4,289. Translated by R. Battison with the assistance of F. Grosjean. Hughlings-Jackson, J. 1878. On affections of speech from disease of the brain. Brain, 1, 304-330. Markowicz, H. 1973. Aphasia and deafness. sign Language Studies, 3, 61-71. Reed, J. 1971. Aphasia and language functioning of the deaf. American Annuls ofthe Deaf, 116, 420-426.

Sarno, J., Swisher, L., & Sarno, M. 1969. Aphasia in a congenitally deaf man. Cortex, 5, 398-414. Tureen, L., Smolik, E., & Tritt, J. 1951. Aphasia in a deaf-mute. Neurology, 1, 237-249. Underwood, J. 1972. Aphasia in the congenitally deafpopdation. Unpublished manuscript, University of Denver.