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[Menière's disease and sudden deafness (author's transl)].

The question is, is there any relation between sudden deafness and Menière's disease? Total hearing loss without recovery is typical of viral infection. In cases of sudden deafness some hearing rest generally remains. It can be the first symptom of Menière's disease, like the first attacks of vertigo which may occur without deafness. Measurements of stapedius reflex threshold and Békésy audiogram, especially the difference between the impulse tone and continuous tone indicate an inner hair cell damage and are helpful in the diagnosis of sudden deafness. But pathological adaptations are seen in cases of sudden deafness and also in Menière's disease. A decrease in the distance between the hearing threshold and the reflex threshold is a further indication of inner ear damage. The independence of the stapedius reflex threshold up to a hearing loss of 60 dB seems to be an indication for the different function of both types of hair cells. the reflex is supposed to be dependent only on the presence of the inner hair cells because the reflex threshold increases as soon as an inner hair cell damage of about 60 dB occurs. Clinical and audiological findings reveal no differences in sudden deafness and Menière's disease.

Audiometry

Autosomal recessive non-syndromal progressive sensorineural deafness in childhood. A separate clinical and genetic entity.

In a family with 11 children, three sibs (two boys and a girl) show progressive sensorineural deafness, first noticed at ages 4, 7 and 11, respectively. The progression of deafness was registered in each of these sibs over a period ranging from 9 to 15 years. The speech perception has simultaneously diminished, as observed also in the case of recruitment. The parents and the other sibs in this family have normal hearing. The parents were demonstrably consanguineous, and autosomal recessive transmission is therefore postulated. A syndromal diagnosis could not be established because general physical, neurological ophthalmological and haematological examination as well as urinalysis revealed no associated characteristics. A syphilitic infection was therefore also excluded as a possible cause. Pendred's syndrome was excluded by means of a potassium perchlorate test. It is believed that these three sibs are suffering from autosomal recessive non-syndromal progressive sensorineural deafness - a type of deafness hardly mentioned in the literature. Several authors differentiate this type of deafness from autosomal recessive early-onset neural deafness.

Adolescent

Extremity of semantic differential ratings in deaf and hearing subjects.

A comparison was made separately by sex of 19 deaf and 19 hearing Ss on mean semantic differential ratings of contradictory concept-pairs. There were nine deaf males, 10 deaf females, 11 hearing males, and eight hearing females with mean ages of 23.00, 21.90, 20.73, and 19.75, respectively. It was hypothesized that the deaf would make less extreme ratings of concept-pairs than hearing Ss and that they would shift their ratings of contradictory concept-pairs closer to the mean of the original ratings of the single concepts comprising the pairs. It was found that both predictions were true only for deaf males. Deaf females did not differ from hearing Ss.

Adult

Vibratory perception by deaf children.

Audiologists and educators of the deaf have become interested in vibratory perception of speech because: (1) research suggests that profoundly deaf children feel rather than hear amplified speech through their ears, and educators must help them to use this acoustic (vibratory) speech information; and (2) vibratory transducers already are used in many classrooms for deaf children to provide supplementary speech input, and educators must learn how to present vibratory stimuli most effectively for perceptual learning. Vibration provides mainly the gross time-intensity information in speech. This paper describes the variety of speech cues that are available through the vibratory sense, for example, number of syllables; syllable rate, stress, and onset/decay; pause; and roughness. An optical analog (tracings from a storage oscilloscope) is used to show the type of information available to deaf persons through vibration. Deaf children are able to perform not only closed-set recognition tasks but also can learn to identify general characteristics of vibratory speech patterns. Although vibratory perception alone is limited for communication, vibratory input can serve as a supplement to lipreading and also as a speech-training aid for profoundly deaf persons.

Bone Conduction

Family therapy in treatment of the deaf: a case report.

Deaf patients with psychological problems have developmental handicaps and clinical characteristics that reduce the effectiveness of traditional modes of psychotherapy. Attempts have been made to utilize individual and group therapy, but family therapy has been largely overlooked as a method of alleviating problems of the deaf. Clinical and research writings provide us with rich insights into the family dynamics of the deaf. These data suggest to the authors that the problems of deaf individuals are largely related to family problems, and therefore merit a family orientation as the focus for treatment. This paper describes an attempt to apply family therapy with a range of deaf patients over a period of two years. From a review of their work, the authors conclude that family therapy can be effective, particularly in the treatment of deaf adolescents and children.

Adolescent

[Different forms of deafness in Treacher - Collins-Syndrome, shown on a family over three generations (author's transl)].

A Treacher Collins-syndrome has been followed up in a family over 3 generations. We found a change in deafness from grand parents- and parents-generation to the third one. The first two showed an inner ear deafness, the latter the well-known conduction deafness. The phenotype becomes more and more extensive in the following generations. Simultaneous with this development the deafness becomes more typical. In theory the inner ear deafness could either be explained as the final stage of a long standing conductive deafness or it is part of an incomplete developed syndrome.

Abnormalities, Multiple

[Sudden deafness in connection with mumps (author's transl)].

The authors report three cases of sudden deafness due to mumps. On the first and second case (children) the deafness was severe perceptive and bilateral; in the first case the perceptive deafness occurred on the eight day while in the second case the forteenth day from the onset of mumps. On the third case (male adult) the deafness was unilateral, sudden with degeneration of vestibular, which occurred during virumia of mumps. The characteristic of this case is that the total deafness of right ear is associated with right parotitis and right orchitis. According to bibliography the pathogenesis, the time of deafness, appearance and the degree of hearing loss are discussed.

Adolescent

Neonatal screening for detection of deafness.

To assess various methods of early detection of deafness, a longtudinal study of infants born in the Jerusalem area was performed. The 17,731 newborns were tested by an acoustic signal generator hearing test (Apriton), and the "at risk" for deafness register was applied to them. Twenty-five children were identified as deaf, 14 with severe or profound hearing loss and 11 with moderate or moderately severe loss. The screening hearing test did not prove to be sensitive enough for detection of deafness in newborns, and, therefore, is not considered valid for screening purposes. The at risk for deafness register, which in our program covered 20% of the entire newborn population, proved to be to expensive and impractical; a restricted register, including approximately 7% of the newborns, is suggested.

Acoustic Stimulation

The Perrault syndrome: autosomal recessive ovarian dysgenesis with facultative, non-sex-limited sensorineural deafness.

We report three sisters with ovarian dysgenesis; all three and two of their otherwise apparently normal brothers also had moderate to severe sensorineural deafness. Three similarly affected sibships are known, and the total of 14 affected patients includes three males with deafness without gonadal defect, one woman with ovarian dysgenesis without deafness, and ten women with ovarian dysgenesis and deafness. In two families parental consanguinity is known. We conclude that this condition, which we propose to designate the Perrault syndrome, is an uncommon autosomal recessive trait with obligatory ovarian dysgenesis in female homozygotes and facultative deafness in male and female homozygotes. Right bundle branch block and mental retardation may possibly be additional, less common pleiotrophic manifestations.

Adolescent

Relationship of word knowledge to word frequency in young adult deaf students.

This report describes research that has been undertaken to determine the relationship between word knowledge and word frequency (occurrence in print) in a deaf population. A matching test was designed and administered to 162 deaf students entering the National Technical Institute for the Deaf in the summer of 1976. The results of the study indicate that there exists a relationship between word frequency and word knowledge in a deaf population. The reliability and validity of the technique was tested and seems to provide adequate support for this approach to assessing the vocabulary level of deaf students.

Adult

Toward a psychology of deafness?

A large scale study comparing Yugoslavian and American adolescents with normal hearing and with early profound deafness suggests that a relative tendency to impulsivity is a notable characteristic of the deaf group. Despite the conclusiveness of the study overall, there was little intercorrelation among individual subjects on the several test measures employed. A stereotype should be avoided therefore, and the tests viewed as perhaps tapping different personality aspects or avenues of development for what clinically becomes a final common pathway. Comparable and comparative studies of deaf children of deaf parents and of children whose hearing parents have learned manual language early may define how much the tendency is based on qualities of parental discomfort with a deaf child, on the unavailability of language, or on the absence of sound alone.

Adolescent

Deaf and hearing children's use of language describing temporal order among events.

Six experiments are described in which deaf and hearing subjects decided the temporal order of events in picture series and in sentences. The deaf subjects, eight and 11 years old, performed as well as hearing children on a nonverbal picture seriation task. Both deaf and hearing subjects also described most picture series in the natural left-to-right order in which they were shown, and identified the left-hand picture in most series as happening first and the right-hand picture as happening last. In most respects, the deaf children's linguistic performance resembled that of much younger hearing children. Two major results were that deaf children generally used a sequence of simple sentences to describe the events shown in a picture series, and responded to most multiple-clause sentences presented as though the events being described had occurred in the order they were mentioned.

Auditory Perception

Instructed rehearsal strategies' influence on deaf memory processing.

Congenitally deaf subjects were compared with normal-hearing subjects on short-term retention accuracy and correct response latency. The subjects paced themselves through serial lists of consonant letters six- or seven-items long. Presentation of each list was followed by a position-probe test requiring the subjects to specify where in the list a particular letter had appeared. The subjects were first observed while generating their own input strategies (free strategy). In subsequent sessions they adopted instructed rehearsal strategies involving primary and secondary memory components. Overall, the normal-hearing subjects were more accurate and responded faster than the deaf subjects. Instructing rehearsal strategies resulted in immediate gains on these measures for both groups. For both measures the deaf subjects became at least as proficient as the normal-hearing subjects had been under free strategy. The patterns of correct response latencies for the groups revealed strikingly different comparisons for primary and secondary memory. Following strategy instruction, latencies for the terminal list items never differed for the two groups, indicating that primary memory in the deaf is fully intact. However, the deaf responded slower on the first items of the list, indicating secondary memory deficiencies.

Adolescent

Paget's disease and sensori-neural deafness: temporal bone histopathology of Paget's disease.

Four cases with Paget's disease of the temporal bone are presented to illustrate the pathogenesis of the associated deafness. One case illustrates the combination of severe deafness due to bilateral otosclerosis with probably asymptomatic bilateral Paget's disease. One case with advanced Paget's disease presents features to explain early stages of sensori-neural deafness before actual cellular invasion of the inner ear. One case of profound deafness due to Paget's disease presents a different stage of cellular invasion of the inner ear by the disease on each side. One case illustrates invasion of the internal auditory meatus by Paget's disease with infiltration of the acoustic division of the nerve and profound deafness.

Adult

The early detection of childhood deafness.

Early commencement of habilitative measures is vital for a deaf child's development. This implies a need for early diagnosis. Some improvement has occurred in recent years, but early diagnosis is still the exception rather than the rule. To determine some of the factors which lead to early diagnosis, a survey of parents of young Australian deaf children was undertaken. This showed that, while the majority of children were suspected to be deaf on the basis of informal observation by parents, relatives or friends, the most consistent early diagnosis came as a result of follow up of infants at risk. Certain factors acted to delay diagnosis. Dissuasion and inappropriate advice from doctors significantly delayed diagnosis in 25% of all cases. Further, over half the hearing-impaired children who were tested in screening programmes were not detected, and the false sense of security given the parents in these cases resulted in long delays before suspicion of deafness (based on informal observation) developed. If early diagnosis of deafness is to become the rule, a purposeful attempt to use risk criteria, coupled with improved screening programmes, will be the most promising avenues to follow. Improved education of the community at large may also assist, but this is a much longer-term aim.

Australia

The attainment of formal operations: a comparison of probability concepts in deaf and hearing adolescents.

In a series of six experiments deaf and hearing adolescents were compared on attainment of formal operations in Piaget's theory as represented by performance on probability problems. Performance of deaf subjects provided a desirable control comparison, since their knowledge of probability concepts was most likely acquired outside school classrooms. The probability problems were usually binary choice situations that required no verbalizations and were designed to illuminate strategies, as well as to indicate conceptual stage. Two experiments, one a long-term training study of over two years' duration, used supplementary preadolescent subject groups. Most of the hearing adolescents and many of the deaf adolescents attained the formal operatory level for probability concepts according to the criteria described by Piaget and Inheldermtheir criteria can be criticized, however, as insufficiently demanding, although they do point up the difficulty of defining the place of computational abilities in formal operationsmresults from several experiments suggested a second and higher level of formal operatory attainment around the ages of 14 to 15. Further theoretical insights were obtained regarding the implicit rules that frequently appeared to guide subjects' choices; a surprising variety of strategies were reported. Analysis of choice tendendies showed consistent avoidance of calculations and selection of the choice with the greatest absolute rather than relative frequency (or area) even when these strategies produced systematic errors. Subjects were generally unable to combine probability information from two different sources. In the long-term training experiment spontaneous improvement was shown by children at formal operatory ages even without training, but not by younger children. Deaf adolescents performed as well as hearing adolescents on easy problems, but they made more errors on several types of more difficult problems; Deaf adolescents were also less consistent than the hearing in use of rules, and in several experiments they showed a two- to three-year performance lag.

Achievement

Recall in acoustically similar and dissimilar letters by normal and deaf subjects.

Stimulus matrices with 4 X 3 letters from an acoustically similar and dissimilar alphabet were presented tachystoscopically for 5 secs to 41 Ss divided into three groups, i.e. normal and deaf apprentices and deaf children. Each S was administered 42 stimulus matrices. While the difference in the number of errors between the acoustically similar and dissimilar alphabets proved significant in the hearing Ss, it was nonsignificant in the deaf, and in contrast to these two groups, in the children the number of errors with the acoustically similar alphabet was lower than with the dissimilar one. The error matrices indicate a systematic course of errors and their different pattern for the hearing and the deaf Ss. A visual and an articulating code which is reinforced by the length of oral training, may be presumed particularly in the deaf.

Acoustic Stimulation

[About progressive deafness after a detonation and explosion trauma (author's transl)].

A case is reported in which deafness was caused by an explosion trauma during wartime. In an expertise in 1954 a causal relationship between the explosion and the deafness was rejected. Advances in medical technology and research into deafness resulting from acustic trauma led to a revision of the previous decision, therefore in 1978 the deafness was acknowledged as war damage and this statement was confirmed by court judgement. The possibilities of a progressive development of ear or skull trauma due to sound is discussed. The point is stressed that there can be longtime intervals between the trauma and the onset and development of resulting deafness. This fact should induce therapeutic consequences, some of the so far tried approaches in therapy are reported.

Audiometry