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[The acquisition of language through finger spelling in a child with auditory agnosia (author's transl)].

A child with auditory agnosia for verbal and nonverbal sounds was reported. He was born without difficulty and his early development was normal. At the age of 14 months, he suffered from herpes simplex encephalitis and fell in a trance. Soon after he recovered from the illness, he found not to pay any attention to sounds and not to speak any words. He was referred to Teikyo University hospital at 2.5 years of age for the hearing measurement. The average threshold of conditioned orientation reflex audiometry at 500, 1000 and 2000 Hz were 85 dB, while the threshold of auditory brain stem response was 15 dB which is normal. CT scan demonstrated bilateral lesions of superior temporal gyrus. Neurological examination revealed nothing particular except the troubles with hearing and speech. He was made a diagnosis of auditory agnosia. The speech therapy was started at two years old. During the first three years, he was taught how to communicate with gestures and lip reading. However, it was difficult for him to understand the meaning of gestures and lip reading. During these years he could acquire lip reading of simple words. As to environmental sounds, he could respond the telephone-bell and the organ at three years and seven months old, but could not discriminate other sounds. At five years old, finger spelling for his language education was introduced. He appeared to have learned finger spelling more easily and could read and write several letters through finger spelling within a month. One year after introduction of finger spelling, he acquired about 60 words (54 nouns and 6 verbs) and could read words and two-words sentences. However, the speed of his acquisition of language was very slow for his age. Now, he can understand mother's simple instructions and communicate with his mother and his speech therapist through finger spelling, but his responses toward environmental sounds are not stable yet. The course of this patient suggests that language acquisition of children with auditory agnosia might be different from that of deaf children. The prognosis of auditory agnosia in children is controversial. In this patient, the development of language is not good as well as in most previous reports, perhaps because of extensive lesions of language area. The patients with auditory agnosia is frequently mistaken for deaf or mentally retarded children. We emphasize that the early diagnosis and early speech therapy with visual communication are needed for these children.

Agnosia↗

Audio-visual speech perception in schizophrenia: an fMRI study.

Abnormalities in the integration of auditory and visual language inputs could underlie many core psychotic features. Perceptual confusion may arise because of the normal propensity of visual speech perception to evoke auditory percepts. Recent functional neuroimaging studies of normal subjects have demonstrated activation in auditory-linguistic brain areas in response to silent lip-reading. Three functional magnetic resonance imaging experiments were carried out on seven normal volunteers, and 14 schizophrenia patients, half of whom were actively psychotic. The tasks involved listening to auditory speech, silent lip-reading (visual speech), and perception of meaningless lip movements (visual non-speech). Subjects also undertook a behavioural study of audio-visual word identification designed to evoke perceptual fusions. Patients and controls both showed susceptibility to audio-visual fusions on the behavioural task. The patient group as a whole showed less activation relative to controls in superior and inferior posterior temporal areas while performing the silent lip-reading task. Attending to visual non-speech, the patients activated less posterior (occipito-temporal) and more anterior (frontal, insular and striatal) brain areas than controls. This difference was accounted for largely by the psychotic subgroup. Insular and striatal areas were also activated in both subject groups in the auditory speech perception condition, thus demonstrating the bimodal sensitivity of these regions. The results suggest that schizophrenia patients with psychotic symptoms respond to visually ambiguous stimuli (non-speech) by activation of polysensory structures. This could reflect particular processing strategies and may increase susceptibility to certain paranoid and hallucinatory symptoms.

Adult↗

Learned audio-visual cross-modal associations in observed piano playing activate the left planum temporale. An fMRI study.

Lip reading is known to activate the planum temporale (PT), a brain region which may integrate visual and auditory information. To find out whether other types of learned audio-visual integration occur in the PT, we investigated "key-touch reading" using functional magnetic resonance imaging (fMRI). As well-trained pianists are able to identify pieces of music by watching the key-touching movements of the hands, we hypothesised that the visual information of observed sequential finger movements is transformed into the auditory modality during "key-touch reading" as is the case during lip reading. We therefore predicted activation of the PT during key-touch reading. Twenty-six healthy right-handed volunteers were recruited for fMRI. Of these, 7 subjects had never experienced piano training (naïve group), 10 had a little experience of piano playing (less trained group), and the remaining 9 had been trained for more than 8 years (well trained group). During task periods, subjects were required to view the bimanual hand movements of a piano player making key presses. During control periods, subjects viewed the same hands sliding from side to side without tapping movements of the fingers. No sound was provided. Sequences of key presses during task periods consisted of pieces of familiar music, unfamiliar music, or random sequences. Well-trained subjects were able to identify the familiar music, whereas less-trained subjects were not. The left PT of the well-trained subjects was equally activated by observation of familiar music, unfamiliar music, and random sequences. The naïve and less trained groups did not show activation of the left PT during any of the tasks. These results suggest that PT activation reflects a learned process. As the activation was elicited by viewing key pressing actions regardless of whether they constituted a piece of music, the PT may be involved in processes that occur prior to the identification of a piece of music, that is, mapping the complex sequence structure of hand movements onto the sequence of sounds.

Adolescent↗

Cochlear implant results: a comparison of live-voice and videotaped tests.

The Cambridge Cochlear Implant Programme has so far implanted the Ineraid multichannel cochlear implant in 16 profoundly deaf adult patients; there has been a 9-month or longer follow-up period with these patients. We have evaluated these patients by open-set Bamford-Kowal-Bench (BKB) Standard Sentence List testing in two different delivery strategies, live-speaker testing by the same speaker and high-resolution videotaped testing. The performance in lip reading both before and 9 months after implantation has been tested, as well as performance with the implant alone and with the implant in conjunction with lip reading at the 9-month stage. We have compared the performance in these two delivery strategies and have found a significantly better performance in the live-speaker tests that is attributable to slower and perhaps more sympathetic delivery. We have also found evidence of a ceiling effect in the performance of the implant with lip reading in the live-speaker mode and, of greater importance, a floor effect in the performance of the implant alone with the videotaped test. These results and the implications for a complementary role of these two test-delivery modes are discussed.

Cochlear Implants↗

Auditory brainstem implant: part I. Auditory performance and its evolution over time.

OBJECTIVE: Evaluation of auditory performance and its evolution over time in patients with the auditory brainstem implant. STUDY DESIGN: Prospective study. SETTING: Tertiary referral center. PATIENTS AND METHODS: Between May 1996 and April 2000, 14 patients with neurofibromatosis type 2 underwent implantation with a multichannel auditory brainstem implant. Auditory performance data were obtained in 13 patients who had used their device on a regular daily basis for 1 to 41 months (average 19 months). Hearing evaluation was based on the results of four tests (vowel confusion, consonant confusion, Freiburger numbers, and speech-tracking test), which were performed with and without lip-reading at regular intervals after device activation. RESULTS: 12 patients received auditory sensation through the auditory brainstem implant immediately after device activation. In one patient, because of postoperative electrode migration, device activation was not successful. In this case, after the electrode array was repositioned, activation was successful. The results of the audiovisual mode 2 weeks after device activation revealed a lip-reading enhancement above the chance level in about 50% of the patients in the vowel confusion and speech-tracking tests and in 70% of the patients in the consonant confusion test. Lip-reading enhancement improved within the first 6 months and then entered a plateau phase, which was more prominent in the monosyllabic vowel and consonant tests. In the auditory alone mode, more than half of the patients showed their first positive result in the vowel test 3 months after device activation, but it took about 6 months until half of the patients revealed a result above the chance level in the consonant and Freiburger numbers tests. Open set speech recognition in the auditory alone mode (in the speech-tracking test) was not common and happened relatively late (within 1 year or later). DISCUSSION AND CONCLUSION: Although auditory sensation appeared immediately after device activation, a period of 6 months was necessary for relearning and adaptation of the central auditory system to the altered form of auditory information presented by the auditory brainstem implant.

Adult↗

Cochlear prostheses in use: recent speech comprehension results.

Speech-comprehension tests were performed with several deaf volunteers who were equipped with different cochlear prostheses. These prostheses work with inductive transcutaneous signal transmission and contain a multi-channel scala tympani electrode or, in two cases, a disc electrode situated external to the cochlea in the round window niche. The external portable single-channel speech-processor/transmitter used is the same in all cases. The comprehension (stimulation only, no lip reading) of numbers between 1 and 99 on first contact with the stimulator and therefore without any training was around 38% showing that through well designed single-channel stimulation of the auditory nerve it is possible to generate hearing sensations sounding very much like nature hearing impressions. Some open speech comprehension was reached: Through the help of the stimulator, without lip reading, between 60 and 100% of sentences unknown to the completely deaf volunteer, spoken only a little more slowly than normal, are correctly understood. The percentage for isolated unknown words is 60%. Also an important improvement of word comprehension can be achieved through additional stimulation as compared to unaided lip reading. It appears that the most recent design of our cochlear prosthesis can already be regarded as a useful aid for the deaf.

Adult↗

The Chorimac-12. A multichannel cochlear implant for total deafness. Description and clinical results.

More than 90% of cases of patients suffering from acquired or congenital deafness may be provided with some sound sensation owing to the electrical stimulation of the few cochlear nerve fibers that are still present in the deaf cochlea. In order to obtain some speech discrimination without lip reading it is necessary to elicite different frequency sound sensations. Thus electrically proofs compartments must be performed all along the cochlear keyboard. The placement of 12 intra-cochlear electrodes is greatly facilitated by using an electrodes-bearer which is introduced through a very simple and classical surgical procedure. The indications for implanting depend on undiscussible criteria (total deafness, auditory response to electrical stimulation of the round window, patient's motivation). Clinical results consist of psychoaffective improvement and auditory possibilities. All patients are able to discriminate homosyllabic words in closed lists without lips reading, but the percentages of these performances greatly vary from one patient to the other. Some patients, generally suffering from acquired total deafness, are able to discriminate words or sentences without reference list and without help of lips reading. These results depend on the patient's socialization level, on the pre or post-lingual status of the deafness, on an eventual cochlear ossification, and on the value of several pre operative electro physiological data. Associated handicaps (autism, blindness, tetraplegia) are not contra indications and often represent supplementary reasons for implanting. The value of these post-operative results may be predicted owing to a series of electrical pre-operative tests and clinical data. Thus it is possible to determine the respective indications of multi and single channel cochlear implants.

Adolescent↗

Cross-modal integration and plastic changes revealed by lip movement, random-dot motion and sign languages in the hearing and deaf.

Sign language activates the auditory cortex of deaf subjects, which is evidence of cross-modal plasticity. Lip-reading (visual phonetics), which involves audio-visual integration, activates the auditory cortex of hearing subjects. To test whether audio-visual cross-modal plasticity occurs within areas involved in cross-modal integration, we used functional MRI to study seven prelingual deaf signers, 10 hearing non-signers and nine hearing signers. The visually presented tasks included mouth-movement matching, random-dot motion matching and sign-related motion matching. The mouth-movement tasks included conditions with or without visual phonetics, and the difference between these was used to measure the lip-reading effects. During the mouth-movement matching tasks, the deaf subjects showed more prominent activation of the left planum temporale (PT) than the hearing subjects. During dot-motion matching, the deaf showed greater activation in the right PT. Sign-related motion, with or without a lexical component, activated the left PT in the deaf signers more than in the hearing signers. These areas showed lip-reading effects in hearing subjects. These findings suggest that cross-modal plasticity is induced by auditory deprivation independent of the lexical processes or visual phonetics, and this plasticity is mediated in part by the neural substrates of audio-visual cross-modal integration.

Adult↗

Patient performance with two types of multiple electrode intracochlear implant.

The functional results of 9 persons implanted with the Symbion Ineraid and 8 persons implanted with the Nucleus cochlear implant with the F0, F1, F2 coding strategy are reported. All patients were postlingually totally deaf, the patients implanted with the Symbion Ineraid were all above 40 years of age, while the Nucleus patients were with one exception under 40 years of age. The CID Everyday Sentences Test, the Helen Test and the Speech Tracking Test showed that all patients improved their communication skills when sound through the implant was added to lip-reading. The patients' ability to understand without lip-reading was tested with the CID everyday sentences test, with the Helen test, with spondees, with three-choice vowels and with a consonant confusion test. These showed that all 9 Symbion Ineraid patients achieved some degree of open speech understanding. Four of the 8 Nucleus patients achieved some degree of open speech understanding. Difference limen tests for intensity and frequency indicated that 2 of the Nucleus patients who did not achieve open speech understanding probably had extensive cochlear nerve damage, while the remaining 2 Nucleus patients without open speech understanding had good frequency and intensity discrimination.

Acoustic Stimulation↗

She won't look at me.

Early-onset hearing impairment is a common disability in the United States. Persons with hearing loss, whether they use American Sign Language or lip-read, must look at those with whom they are speaking. Lip reading is not a reliable method of communication for most deaf persons. Reading and writing also limit the amount of communication between health care providers and deaf patients. The best way to communicate with most deaf persons is through a qualified American Sign Language interpreter. This paper discusses communication with deaf persons and ways in which health care providers and hospitals can improve their interactions with deaf patients.

Communication Devices for People with Disabilities↗

Seeing to hear better: evidence for early audio-visual interactions in speech identification.

Lip reading is the ability to partially understand speech by looking at the speaker's lips. It improves the intelligibility of speech in noise when audio-visual perception is compared with audio-only perception. A recent set of experiments showed that seeing the speaker's lips also enhances sensitivity to acoustic information, decreasing the auditory detection threshold of speech embedded in noise [J. Acoust. Soc. Am. 109 (2001) 2272; J. Acoust. Soc. Am. 108 (2000) 1197]. However, detection is different from comprehension, and it remains to be seen whether improved sensitivity also results in an intelligibility gain in audio-visual speech perception. In this work, we use an original paradigm to show that seeing the speaker's lips enables the listener to hear better and hence to understand better. The audio-visual stimuli used here could not be differentiated by lip reading per se since they contained exactly the same lip gesture matched with different compatible speech sounds. Nevertheless, the noise-masked stimuli were more intelligible in the audio-visual condition than in the audio-only condition due to the contribution of visual information to the extraction of acoustic cues. Replacing the lip gesture by a non-speech visual input with exactly the same time course, providing the same temporal cues for extraction, removed the intelligibility benefit. This early contribution to audio-visual speech identification is discussed in relationships with recent neurophysiological data on audio-visual perception.

Cues↗

Results of the MXM Digisonic auditory brainstem implant clinical trials in Europe.

OBJECTIVE: To assess the potential benefit of the MXM auditory brainstem implant for patients with neurofibromatosis type 2. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral centers. PATIENTS: Fourteen patients with neurofibromatosis type 2 and bilateral acoustic neuromas underwent implantation with the MXM auditory brainstem implant during surgery to remove the second-side tumor. RESULTS: There were no complications related to the auditory brainstem implantation. Auditory sensations were present for 12 of 14 patients (86%). Global results indicated an improved quality of life for the patients receiving auditory sensations, in part because of their auditory orientation within the environment. Eighty-nine percent of patients tested with an open-set sentence test demonstrated enhancement of speech understanding as a result of lip-reading improvement when auditory brainstem implant sound was combined with lip-reading. A few patients (36%) had some speech understanding in sound-only mode. One patient was able to have limited phone conversations. CONCLUSION: These results indicate that significant auditory benefit can be derived from the MXM auditory brainstem implant.

Adolescent↗

Benefits of cochlear implantation in early-deafened adults: the Toronto experience.

OBJECTIVE: To present the results of a survey administered to a group of early-deafened cochlear implants adults and to report the level of perceived benefit. DESIGN: Prospective. SETTING: Large tertiary referral centre. METHOD: A 47-item questionnaire designed to evaluate cochlear implant use and benefit was sent to 42 early-deafened adult cochlear implant users. The questionnaire can be divided into seven subcategories: time of use, associated symptoms, communication, employment status and function, socialization, perceived benefit, and the impact on quality of life. Responses from 30 patients were received. RESULTS: The majority of our patients use their cochlear implant all of their waking hours. The majority of patients continue to depend on lip-reading and hearing as their main mode of communication, although they reported improved lip-reading skills with their cochlear implant. Twenty-three patients (76.7%) were employed. Eleven patients had a change in employment subsequent to cochlear implantation, nine (81.8%) of whom attributed this to their cochlear implant. Our patients als reported greater independence, a greater sense of safety in their environment, and an improved social life. Twenty-nine patients (96.7%) said that they were satisfied with their implant, 28 (93.3%) said that they would go through the same process again, and 27 (90%) said that they would recommend it to a friend in a similar situation. Twenty-nine patients (96.7%) stated that the cochlear implant has had a positive effect on their quality of life. Family and peer support, prior auditory-verbal therapy, and a positive attitude were the most commonly cited factors in successful cochlear implant use. CONCLUSIONS: Early-deafened adult cochlear implant users perceive significant benefit from cochlear implantation. Importantly, family and peer support, prior auditory-verbal therapy, and a positive attitude are considered important factors in maximizing this benefit.

Adult↗

The University of California, San Francisco/Storz cochlear implant program.

Using a four-channel implant system with a vocoder-based processor developed at UCSF over an extensive period of research, clinical trials of the UCSF/Storz device were initiated in February 1985 under the sponsorship of Storz Instrument Company. To date, 13 patients have been implanted with the UCSF/Storz device, 10 of whom have been fitted with their external processor and transmitter and have received at least their initial postoperative evaluations. Of these 10 patients, nine are able to use all four channels of their implant system. The device fitting/adjusting process for these patients has been remarkably easy, requiring only approximately 30 to 60 minutes. Patient results have been extremely good, with eight of the 10 patients obtaining some open-set auditory only speech understanding. Without extensive rehabilitation and training, most patients have demonstrated an improvement in speech reception over time. Lip-reading and tracking results indicate that all patients have attained an enhancement of lip-reading ability with the use of the device, suggesting that improved general communication skills have been provided for each UCSF/Storz patient.

Adult↗

Discovery and expository methods in teaching visual consonant and word identification.

An experiment was conducted to examine the processes involved in lipreading as well as to investigate an optimal approach to teaching lipreading skill. We compared discovery and expository methods of learning to lip-read. Twenty-six college students with normal hearing were trained over 3 days to lip-read consonant-vowel (CV) syllables. The training material consisted of a prerecorded videotape of four different talkers. The task was a forced-choice procedure with feedback. Subjects learned with training, but there was no difference between the two learning methods. As a retention measure, subjects returned 4 weeks later and repeated the training. There were significant savings of the original learning. Three weeks after the retention phase, subjects were tested with a 10-item forced-choice monosyllabic word task. Those subjects who had extensive training on CV syllables did no better on identifying the monosyllabic words than did a control group of 9 subjects with no training. Nevertheless, performance for all three groups (discovery, expository, and no training) improved during training in the word identification task.

Adolescent↗

The bionic ear: principles and current status of cochlear prostheses.

The field of cochlear prostheses is reviewed, emphasizing the neurophysiological principles necessary for the development of a successful cochlear prosthesis. Pertinent auditory physiology is reviewed, and four conditions are proposed which, if met, should result in speech recognition by the patient with an implant. These conditions are: The surviving neural population must be adequate over the frequency distribution required to deliver the necessary information for speech discrimination. The central neural processing pattern for decoding and recognizing speech must have been established and still persist. The processing of the acoustic signal by the auditory system up to the level of the prosthesis interface must be understood and predictable from the acoustic stimulus. The relationship between the response patterns of the neural elements and the electrical stimulus must be well documented and controllable. The degree to which these conditions can presently be met is discussed, with some suggestions for future development. The function of the cochlear prosthesis is separated into a signal processing section and an electrical stimulus section. Two signal processing strategies are analog processing of the acoustic signal and speech feature extraction from the acoustic signal. Four possible electrical stimulation strategies are direct analog stimulation from the signal processor, pulse amplitude modulation, pulse width modulation, and stimulation designed to optimize the neural responses to electrical stimulation. Some of the present generation cochlear prostheses, including five approved by the FDA as investigational devices, are discussed according to this classification. The clinical results of testing these devices as aids to lip reading and as stand-alone speech reception aids are compared. All are a potential aid to lip reading. Some prosthesis designs have been implanted in patients who then showed exceptional open set speech discrimination. These exceptional patients have used both single channel and multichannel devices and devices with both analog processing and speech feature extraction strategies. These results are encouraging. More consistent and better speech reception is anticipated for the future as this field develops.

Auditory Pathways↗

Direct and indirect therapy for word sound deafness.

This paper evaluates two therapy programmes conducted with PK, an aphasic individual with word sound deafness. The first aimed directly to improve discrimination skills, using minimal pair tasks supported with lip reading. Disappointingly, there were no changes on discrimination tests after this therapy, even when PK could benefit from lip reading information. The second, indirect, programme of therapy aimed to change the communication behaviours of PK's wife. Strategies such as writing and simplifying information were modelled and practiced. A detailed information booklet, outlining the target strategies and explaining PK's main strengths and weaknesses supported the programme. Evaluation of this therapy involved pre- and post-therapy analysis of interactions between PK and his wife, with biographical questions forming the basis of the interactions. There were several changes after therapy on this measure. The number and length of communication breakdowns were reduced, and more questions were answered accurately. This study suggests that, in some cases of severe word sound deafness, indirect therapies may be most effective.

Aged↗

[Cortical deafness following bilateral hypertensive putaminal hemorrhage].

A case of cortical deafness is reported, which was caused by two episodes of bilateral hypertensive putaminal hemorrhage. Such a case has not appeared in literature up to this time. The patient was a right-handed, 69-year-old man, admitted to us after the stroke with right hemiparesis and disturbance of consciousness. CT scans showed right putaminal hemorrhage. After recovery of consciousness, the patient complained of hearing difficulty of the right ear. The right hemiparesis improved and he was followed ambulatorily after two months hospitalization. He returned to his work and was able to drive a car. His hearing difficulty of the right ear progressed and became stone deaf six months after onset of the initial stroke. Two years after the first stroke, he was attacked by the second stroke with left hemiparesis. CT scans showed left putaminal hemorrhage larger than the previous one in the right. When the patient recovered consciousness, he complained of total deafness. Audiometry test showed that hearing was lost completely to both high and low notes, and there was absence of bone conduction bilaterally. Auditory brain stem response test revealed normal pattern from wave I through wave V bilaterally. Middle latency response showed normal component Po on the right ear and it was absent on the left. The component Pa was not identified on both ears. He showed skill and reliance on lip reading two years after the onset of the complete deafness. Now he can speak fluently and communicate with lip reading and written language, although no improvement of the complete deafness being noticed.(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗