Electrophysiological registration of phonological perception in the subthalamic nucleus of patients with Parkinson's Disease.

Phonological processing is usually associated with the activation of cortical areas, especially in the left cerebral hemisphere. This study examined if phonologically elicited evoked potentials can be recorded directly from the subthalamic nucleus in patients with Parkinson's Disease (PD). Seven PD patients who had undergone implantation of deep brain electrodes for the stimulation of the subthalamic nucleus were included. Local field potentials were recorded in a pre-attentive auditory phonological task, an attentive auditory phonological discrimination task, and a word recognition task. Auditory evoked potentials related to phonological, but not lexical processing, could be demonstrated in the subthalamic nucleus for all three tasks. Only minor changes were found after levodopa administration. This study demonstrates that the subthalamic nucleus is involved in early phonological perception, which puts the subthalamic nucleus in a position to modify phonological perception in a larger cortico-subcortical network.

Speech intelligibility of children with unilateral cleft lip and palate (Dutch cleft) following a one-stage Wardill-Kilner palatoplasty, as judged by their parents.

This study determined the intelligibility (words, sentences and story telling) of 43 children (mean age 4.9 years) with unilateral cleft lip and palate who received a Wardill-Kilner palatoplasty, as judged by their parents and determined the influence of age and gender. A comparison with normative data for intelligibility of 163 Dutch speaking children was made. Each child and the parents completed The Dutch intelligibility test. Measures reported include group mean intelligibility percentages for words, sentences and story telling for children aged 2.5-5.0 and 5.0-7.6 years. A significant correlation between age of the children and intelligibility was measured. No significant gender-related differences were found. An interesting finding is the absence of significant difference in intelligibility percentage between the children with cleft palate and the normative data for story telling. The reported intelligibility percentages provide important prognostic reference information for surgeons who perform palatoplasty and speech pathologists who assess the speech production of children with cleft palate.

Speech and language in Simpson-Golabi-Behmel syndrome: a case report.

Simpson-Golabi-Behmel syndrome (SGBS) is an X-linked disorder caused by a mutation of the glypican-3 gene. The physical characteristics associated with SGBS have been documented in several papers, but information on the behavioral phenotype is scarce. We report on the speech and language characteristics in an 8 year-old-boy with SGBS. The boy performed very well on formal language tests but he also demonstrated poor pragmatic skills. Speech was characterized by a distorted articulation, distorted resonance, fluency failures, and a stereotype prosody. Many more case descriptions are necessary to delineate the full range of the behavioral phenotype of SGBS.

Expressive language in persons with Prader-Willi syndrome.

The aim of the present study was to add to the delineation of the prevalence and nature of language difficulties in Prader-Willi syndrome (PWS). Subjects of this study were 32 Dutch speaking individuals (20 males, 12 females), with chronological ages ranging from 4;6 to 29,5 and total IQ ranging from 40 to 94. Spontaneous speech samples were subjected to a consensus orthographic transcription and analysed by means of TOAST, a Dutch standardized diagnostic instrument to investigate spontaneous language production. It was found that expressive language is usually problematic in PWS. Clearly, different linguistic levels are affected and some levels are more prone to difficulty than others. In general morphosyntax appears to be poor, whereas phonology is fairly good. Difficulties with vocabulary and/or pragmatics may be present as well. Considerable inter-individual variation was found as to the severity of the language problems.

Disfluency in Tourette syndrome: observational study in three cases.

It is often stated that stuttering is a common speech disorder in individuals with Tourette syndrome (TS). It has also been suggested, however, that the fluency failures observed in people with TS do not completely conform to the classic pattern of stuttering. The present paper describes the results of an analysis of the speech patterns of three individuals with TS. A picture emerged that bears some resemblance to stuttering, cluttering, and palilalia but that is also different from each of these disfluency types.

Effect of cleft type on overall speech intelligibility and resonance.

The main purpose of this study was to measure and compare the effect of an unilateral cleft lip and palate (UCLP) and a bilateral cleft lip and palate (BCLP) on overall speech intelligibility, nasalance and nasality. An additional objective was to compare the nasalance and nasality patterns of cleft palate children with the available normative data for normal children. The subjects were 37 children with an UCLP (19/37) or a BCLP (18/37) with normal cognitive and motor functions and normal hearing levels. All subjects had a non-syndromic cleft, followed the same surgical protocol and did not undergo secondary pharyngeal surgery. To measure and compare the effect of cleft type, objective and subjective assessment techniques were used. For the objective assessment of nasal resonance the nasometer and the mirror-fogging test were used. Nasalance scores were obtained, while patients produced sounds, read three standard passages or repeated sentences focusing on specific consonants. A perceptual evaluation of speech, the Gutzmann (1913) test and the tests of Bzoch (1989) were used as subjective assessment techniques. The perceptual assessment of the overall speech intelligibility was based on the spontaneous speech and reading or repeating of the nasometric sentences. These samples were judged by three speech pathologists using a 4-category nominal scale. A subjective test (Morley, 1945) was used to evaluate the velopharyngeal mechanism. The findings of the present study show no statistically significant differences for nasalance values, nasality and overall speech intelligibility between the UCLP and BCLP children. Significant differences were found between the data of the normal group and the cleft palate children. These differences included nasalance values as well as nasality data and overall speech intelligibility.

Neurogenic stuttering versus developmental stuttering: an observer judgement study.

It has been claimed by some but rejected by others that neurogenic stuttering has unique features that distinguish this type of dysfluency from developmental stuttering. An experiment is reported in which a panel of professionals was presented at random speech samples from four developmental and four neurogenic stutterers and was asked to classify them accordingly without any patient knowledge. Results support the view that based on the patient's verbal output only it may sometimes be difficult to distinguish between neurogenic and developmental stuttering. Educational outcomes: As a result of this activity, the reader will be able (1) to define acquired stuttering and to list different types of acquired stuttering and (2) to discuss the resemblance between neurogenic stuttering and developmental stuttering.

Clinical evaluation of conversational speech fluency in the acute phase of acquired childhood aphasia: does a fluency/nonfluency dichotomy exist?

Traditional neurologic tenets claim that the clinical picture of acquired childhood aphasia is nonfluent irrespective of lesion location. In the past 20 years, however, several case studies have shown that fluent aphasic patterns can be observed in children with acquired childhood aphasia. But the question remains open as to whether the pattern of their speech characteristics is similar to the one described in adult aphasics as studies addressing spontaneous speech fluency characteristics in larger series of children with acquired childhood aphasia are scarce. The objective of this study was to investigate whether an analysis of spontaneous speech fluency as has previously been performed in adult aphasics by other investigators would also yield two distinct groups of aphasic children and, if so, whether the distribution of the different speech characteristics in both groups would reflect the rank order found in adults, that is, whether nonfluent verbal output characteristics would predominate in one group and fluent features in the other. Audiotaped and videotaped recordings of 24 cooperative children with acute acquired childhood aphasia unselected for age, gender, etiology, and aphasia severity ratings were analyzed according to 10 different speech characteristics. A cluster analysis (two-means clustering) was performed to seek the existence of two distinct groups of aphasic children. Results were confirmed, and exact P values were computed with Mann-Whitney U-tests. A two-means clustering created two distinct classes. Mann-Whitney U-tests ranked the speech characteristics according to their discriminating power between clusters. Comparing this rank order with the one previously found in adults revealed a high correlation (Spearman's rank correlation: r = .915, P << .005), thus indicating that the clusters we found were highly similar to the adult clusters. Thus, the use of the speech variables proposed to evaluate adult aphasic spontaneous speech enabled us to demonstrate a fluent/nonfluent dichotomy in a childhood aphasic population as well. This study shows that the traditional views on the uniformity of the clinical picture of acquired childhood aphasia are obsolete. Our findings corroborate data issued from several case reports of fluent acquired childhood aphasia and from the few studies focusing on speech fluency in acquired childhood aphasia, which all point to the existence of an adultlike heterogeneity of childhood aphasic syndromes. Current clinical evidence no longer supports the hypotheses of equipotentiality and progressive lateralization but favors the notion that the anatomic substrate for language representation in the child is similar to that in adults, even in young subjects.

Speech patterns in children with velo-cardio-facial syndrome. Two case studies.

The purpose of this study was to document the articulation patterns in children with velo-cardio-facial syndrome. The subjects were 2 children with this syndrome, aged 6 years 6 months and 3 years 11 months. Phonetically transcribed speech samples collected by means of a picture naming test formed the basis for this study. The speech samples were subjected to two types of analyses: independent analyses describing the children's sound productions regardless of their relation to the adult targets and relational analyses comparing the children's productions with the adult standard form. It was found that the articulatory problems in velo-cardio-facial syndrome are not simply due to a delay in speech sound acquisition. Articulation errors found in this syndrome are uncharacteristic of normal speech development. Whether errors are syndrome-specific awaits further investigation.

Aerodynamic and acoustic characteristics of voice in Prader-Willi syndrome.

Prader-Willi syndrome (PWS) is a multisystem disorder caused by DNA abnormalities involving chromosome 15. Major characteristics are infant hypotonia, hypogonadism, mental retardation, a short stature, atypical facial appearance, and the onset of obesity due to insatiable hunger in early childhood. Also, speech and language abnormalities have been reported including voice disorders. These have seldom been studied in detail, however. This paper reports the results of an acoustic and aerodynamic investigation of the voice in 22 individuals with PWS. Two age groups were distinguished, a group of children [chronological age (CA) 6 years, 7 months through 11 years, 7 months; total intelligence quotient (TIQ) 40-88] and a group of adolescents and adults (CA 17 years, 1 month through 29 years, 5 months; TIQ 41-94). Both aerodynamic and acoustic parameters were obtained and compared with normative data from the Belgian Study Group on Voice Disorders. It was found that voice difficulties do commonly occur in individuals with PWS including impairment of frequency levels, voice quality, and poor aerodynamic capabilities.

Public awareness of stuttering in Shanghai, China.

This study reports the results of an investigation of knowledge and public awareness of stuttering among laypersons in Shanghai, China. A total number of 1968 respondents answered a questionnaire including questions about prevalence, onset, gender distribution and occurrence in different cultures, cause, treatment, intelligence and heredity of stuttering. Stuttering appears to be a disorder that most people in the Shanghai area are familiar with, and for several aspects a considerable number of respondents held views that are in line with current knowledge about stuttering. For some aspects though knowledge is limited, indicating a need for disseminating scientific information on stuttering. The results of the present study are compared with a similar Belgium study.

Physical appearance and voice in male-to-female transsexuals.

It has been suggested that sex judgments of male-to-female transsexuals based on the voice may be influenced by the physical appearance of the clients. To explore this hypothesis, a listener experiment was designed in which a panel of 22 laypersons and 22 students in speech-language pathology rated the "femaleness" of fourteen male-to-female transsexuals from video-recorded speech samples in three modes of presentation: auditory-only presentation, visual-only presentation, and audiovisual presentation. Results indicate that appearance and voice are indeed interacting factors. Ratings from the auditory-only presentation were significantly lower than ratings from the audiovisual presentation and ratings from the visual-only presentation were significantly higher than those from the audiovisual presentation. It follows that the success of voice training in male-to-female transsexuals is not solely dependent on vocal characteristics and that speech pathologists should consider incorporating physical appearance as a treatment and outcome variable in the voice training of male-to-female transsexuals.

Voice problems in female-to-male transsexuals.

Voice therapy with the aim of raising a client's fundamental frequency is a common practice in the reassignment of male-to-female transsexuals. In female-to-male transsexuals on the other hand voice therapy is usually not considered since the administration of androgens is assumed automatically to change the voice in the direction of the desired sex. The present study examined whether the voice change in female-to-male transsexuals is indeed as straightforward as it is assumed. Results from a sample survey involving 16 female-to-male transsexuals and longitudinal data from two clients suggest that the voice change in female-to-male transsexuals is not always totally unproblematic. A voice assessment and some counselling before the hormone therapy are recommended.

Phonosurgery in gender dysphoria.

A particular type of phonosurgery is voice change surgery in cases of gender dysphoria. This paper presents a review of the surgical techniques available for raising or lowering speaking fundamental frequency. A cricothyroid approximation to raise the vocal pitch is the most common procedure. This procedure results in an increase of about one octave immediately postoperatively and about half an octave after stabilization of the voice.

Dysfluency and phonic tics in Tourette syndrome: a case report.

Tourette syndrome, a condition first recognized in 1825, is characterized by the presence of multiple motor tics and one or more phonic tics. Individuals with Tourette syndrome may also demonstrate fluency failures in their speech. This study investigated the disfluencies and phonic tics in an 18-year-old affected male before and after a three week period of speech therapy. It was found that the speech pattern displayed by this subject did not completely conform to the classic pattern of stuttering but did bear more resemblance to cluttering. A limited number of therapy sessions resulted in a significant improvement of speech.

Speech patterns in Kabuki make-up syndrome: a case report.

The case of a girl aged 3 years and 8 months with Kabuki make-up syndrome is reported. At presentation, she had normal cognitive functioning, and she also had a history of otitis media, a submucous cleft palate, and some hypotonia. Language testing showed normal receptive skills and good expressive vocabulary but poor morphosyntactic abilities. Speech analysis showed that she was capable of producing most of the sounds of her native language but demonstrated high variability in production of the sounds. In addition, she inconsistently simplified words by application of several phonologic processes. Possible explanations for the communication problems demonstrated are discussed.

Communication problems in Turner syndrome: a sample survey.

Turner syndrome is a condition first recognized in 1938 with an incidence estimated at 1/2500 female births. It is known to result from a missing X chromosome and has as its main features a short stature, ovarian dysgenesis, neck webbing, congenital peripheral lymphedema, coarctation of the aorta, cubitus valgus, dysplastic nails, and pigmented nevi. In addition, psychosocial difficulties including communication disorders may occur. While the physical characteristics of Turner syndrome have been well documented, information on the communication problems in Turner syndrome is scarce. This study reports the results of a sample survey in 128 girls with Turner syndrome ranging in age from 2.4 to 58.8 years. Results are presented on the occurrence and nature of speech and language problems (voice disorders, articulation problems, stuttering, and delayed language development), on the presence of learning disabilities, and on educational history.

Public awareness of stuttering.

The results are reported of a questionnaire study into the awareness of stuttering of lay persons in part of Belgium. 1,362 subjects were interviewed. Questions pertained to various aspects of stuttering including prevalence, onset, gender distribution and occurrence in different cultures, cause, treatment, intelligence and hereditariness. Although most respondents were to some extent familiar with stuttering, their overall knowledge of the disorder appeared generally limited.

Progressive dysarthria. Case reports and a review of the literature.

Two patients presenting with progressive dysarthria as the single initial manifestation of a neurodegenerative condition are described. The nature of the dysarthria as well as the additional symptoms that developed in the course of the disorder are very different in these two cases. Nevertheless, neuroimaging findings are strikingly similar and suggest bilateral involvement of posterior inferior frontal lobe structures, mainly in the dominant cerebral hemisphere. The clinical syndrome of these patients can therefore be considered an example of frontotemporal degeneration presenting without dementia or compartmental alteration, at least in the early stages. This broadens the clinical spectrum of frontotemporal degeneration and demonstrates the need for a syndromal subclassification of this nosological entity.