Masako Notoya

Teaching Written Language to Deaf Infants and Preschoolers

The acquisition of oral and written language in six deaf infants is compared from ages one and two years for periods of four and five years. The results indicate: (1) Acquisition of written language is not dependent on oral language; (2) Written language teaching can be initiated at about one year of age; and (3) Written language is easier to learn than oral language. These results suggest that written language instruction in early life helps deaf infants acquire language.

A Case of Pure Word Deafness Associated with Landau-Kleffner Syndrome: A Long-Term Study of Auditory Disturbance

A long-term study of auditory disturbance of a female case who had suffered from pure word deafness associated with Landau-Kleffner syndrome was reported. The patient developed this syndrome at age 4, and we continued the follow-up until she reached 20. The following became clear after the investigation: (1) even by the age of 20 her auditory defect had not improved significantly; (2) from an early stage she could not identify either vowels or consonant-vowel syllables; (3) later she had no difficulty identifying vowels, but her consonant-discrimination score hardly improved; and (4) her problem in consonant identification was unique in that she could discriminate between the voiced and voiceless group but had great difficulty identifying the consonants within each group. These findings led to the conclusion that the patient is unable to recognize short time duration consonants due to an insensibility to loudness and a defect in temporal resolution.

Academic Success of Integrated Hearing-Impaired Pupils Who Had Enrolled in the Early Written-Oral Language Program

Data on academic success were analyzed for 37 hearing-impaired pupils who had been trained by the Written-Oral Language Method before school age. Seventy-one percent of pupils whose average hearing level was less than 89 dB and 77% of pupils whose hearing was 90 to 120 dB succeeded academically. Degree of hearing impairment, speech intelligibility, and gender were not important to their success. Linguistic competence evaluated just before enrollment in a normal educational setting at 6 years of age was the only factor significant to their academic success. However, we propose that the investigation of other factors, IQ and age at the start of preschool training program, must be analyzed in more detail in the future.

Acquisition of Oral and Written Language in Children with Severely Impaired Hearing

The effect of the use of written language (WL) for the training of children with severely impaired hearing was compared with that of oral language (OL). WL training was given by means of a WL card method from the age of 1 (group 1; 6 cases) of 2 (group 2; 2 cases). The children in group 1 acquired 3.8 words in OL and 50.5 words in WL as an average number of receptive vocabulary, while 4.5 words in OL and 50.5 words in WL as that of expressive vocabulary by the age of 1 year and 11 months. In group 2, one case acquired 641 words and six-word sentences in OL and 1,066 words and nine-word sentences in WL for reception, while 401 words and five-word sentences in OL and 1,066 words and six-word sentences in WL for expression by the age of 3 years and 6 months. The other case acquired no word in OL but 676 words and nine-word sentences in WL for reception, while 131 words and four-word sentences in OL and 676 words and six-word sentences in WL for expression by the age of 4. The results would indicate that the training of children with poor hearing using WL from an early period of life is effective for the promotion of the language development.

Parkinson’s disease showing progressive conduction aphasia

Patients with Parkinson’s disease (PD) may develop progressive dementia late in their clinical course. Dementia in PD is mostly related to neuropathological findings of extensive Lewy bodies (LBs), with or without the coexistence of Alzheimer’s disease (AD) pathology. Aphasia has been reported in patients with LB diseases with AD pathology; however, there have been no reports of typical PD patients developing progressive aphasia during their clinical course. We describe a female PD patient who later developed progressive conduction aphasia characterized by phonemic paraphasia and disturbance in repetition of short sentences without disturbance in writing or auditory comprehension. No episodes of fluctuations of attention, memory complaints, or planning errors were observed. She experienced episodes of visual hallucination. Her low scores on the Mini-Mental State Examination suggested impairment of orientation and attention, and her scores on Raven’s Coloured Progressive Matrices test indicated impaired visuospatial functions. However, her cognitive deficits were not sufficiently severe to impair her daily life. Brain magnetic resonance images revealed atrophy of the left superior temporal gyrus and widening of the left sylvian fissure. [18F]-fluorodeoxyglucose positron emission tomography revealed glucose hypometabolism in the left cerebral hemisphere. These findings may be related to conduction aphasia. During the progression of PD lesions, the brainstem LB is assumed to take an upward course, extend to the limbic system, and then extend to the neocortex. Conduction aphasia observed in our patient may be associated with an unusual progression of the LB pathology from the brainstem to the left temporoparietal lobe.

Head Position of Patients with Right Hemisphere Damage during a Visual Search Task in a Large Field

Spatial neglect syndrome with cerebral lesions is characterized by the ina-bility to orient, report, or respond to relevant visual stimuli contralateral to the lesions. In this study, we investigated the pattern of horizontal visual search in a large space; this search was performed by patients with right hemisphere damage caused by cerebrovascular disease. The neck rotation angle and search time in each increment were continuously recorded during the task, and quantitative data of the measurements were collected. Head position during the visual search task in a large space (%) was then calculated. We set angular bands in increments of 5 degrees from the midline on the left and right sides, and calculated the total search time in each angular band. In patients with unilateral spatial neglect (USN), the search time in the angular band of 5 - 9 on the right side was significantly longer (with USN 16.7%, without USN 4.5%, t = 2.52, df = 16, p < 0.05). Furthermore, in patients with neglect in the leftmost end area in a large space, the search time was significantly shorter in the angular band of 1 - 4 degrees to the left (with neglect 13.1%, without neglect 23.7%, t = 2.13, df = 16, p < 0.05), and tended to be long in the angular band of 5 - 9 to the right. The neck rotation angle and pattern during the search task in a large field deviated slightly to the right, and the search time slightly to the left was short. We believe that these results support the rightward deviation of the search pattern and frame of USN patients.

A Study on Visual Search during the Trail Making Test: Analysis Using an Eye Tracker

The Trail Making Test A (TMT-A) is a task related to visual search. Therefore, performance in the TMT-A has a high possibility of being influenced by visual information, such as the arrangement of numbers. The purpose of this study was to measure eye movements during the TMT-A using an eye tracker, to analyze differences in enforcement and trace execution, and to examine appropriate inspection drawings as visual search tasks. Study 1 was performed in 14 young healthy subjects and Study 2 was performed in 6 young healthy subjects. The English and Japanese versions of the TMT-A were administered as usual and the subjects performed the traces in a sitting position. The eye movements of the subjects were measured during the task. We analyzed the combined motion angles of both eyes. We compared the average values (30 Hz/s) of movement in the horizontal (X-axis) and vertical (Y-axis) directions during the normal administration of the English and Japanese version of the TMT-A. We also compared the traces performed by the subjects. There were no differences in tracing or enforcement in the English version of the TMT-A. Especially in the vertical visualization operation, it was shown that the normal enforcement was fewer than the tracing, and almost no up-to-down search was performed. In contrast, the subjects performed visual searches in all directions during the Japanese version of the TMT-A.

Agraphia of Kanji (Chinese characters): an early symptom of sporadic Creutzfeldt-Jakob disease in a Japanese patient: a case report

IntroductionSlowly progressive cognitive decline is the most frequent initial manifestation in MM2-cortical-type sporadic Creutzfeldt-Jakob disease. Agraphia has never been noted in patients with this type of sporadic Creutzfeldt-Jakob disease, however, we report the case of a Japanese patient with sporadic Creutzfeldt-Jakob disease in whom agraphia of Kanji was an initial cardinal symptom.Case presentationA 59-year-old right-handed Japanese woman complained of agraphia of Kanji (Chinese characters) as an initial symptom. A neurological examination revealed mild word-finding difficulty, constructive disturbance, hyperreflexia in her jaw and lower limbs, and bilateral extensor plantar reflexes. An examination of her cerebrospinal fluid revealed increased levels of 14-3-3 and total tau proteins, and abnormal conformation of the proteinase K-resistant prion protein. Diffusion-weighted magnetic resonance imaging showed diffuse hyperintensity in bilateral cerebral cortices. Single-photon emission computed tomography scans revealed hypoperfusion in the left temporal lobe, bilateral parietal and occipital lobes. An analysis of the prion protein gene demonstrated no mutation with homozygous for methionine at the codon 129. We diagnosed our patient with sporadic Creutzfeldt-Jakob disease. Although a histological examination was not performed, it was assumed that our patient could be the MM2-cortical type according to the clinical findings and the elevated levels of 14-3-3 protein in her cerebrospinal fluid. The left posterior inferior temporal area, which was affected in our patient as a hypoperfusion area, is associated with selecting and recalling Kanji characters.ConclusionsFocal signs as an early symptom and hypoperfusion areas in sporadic Creutzfeldt-Jakob disease are critical to recognize initial brain lesions damaged by the proteinase K-resistant prion protein accumulation.

THE EFFECTS OF EARLY MANUAL INSTRUCTION ON THE ORAL LANGUAGE DEVELOPMENT OF TWO DEAF CHILDREN

The speech and language training for deaf children at our clinic is performed using a multisensory method, which consists of reception and expression training for sign language and fingerspelling as well as auditory training, lip reading, and written language training (the Kanazawa Method). We have already reported that acquisition of written language is not dependent on oral language, and that written language is easier to learn than oral language for deaf children. In the present investigation, we analyzed the acquisition of comprehensible and expressive vocabulary in sign language and fingerspelling. The subjects were two children congenitally deaf at levels higher than 105dB. Recorded language samples by the age of 48 months were analyzed. Acquisition of sign language was found to be significantly easier than acquisition of oral language. The development of expressive noun words, function words, and Wh-question words in sign language at the early period was almost equivalent to that of hearing peers, and then the sign language appeared transfer to the oral language. These results suggest that early presentation of sign language with written and oral language is effective in the acquisition of communicative attitudes, function words and interrogative sentences which are most difficult for the hearing-impaired. It was shown that early presentation of sign language with written and oral language serves to promote acquisition of oral language.