[The dosage of botulinum toxin type B in adductor type spasmodic dysphonia]. / Dosierung von Botulinumtoxin Typ B bei spasmodischer Dysphonie vom Adduktortyp.

Today, most patients with severe adductor type spasmodic dysphonia are treated with repeated injections of botulinum toxin type A (BTA). It is known that patients who have been treated for many years and have received a high cumulative dosage may develop antibodies against BTA, making them "resistant" to further injections. For these patients, botulinum toxin type B (BTB, NeuroBloc) is considered to be a new chance to continue the treatment. When changing to BTB, one has to find an "equivalent dosage" which is estimated for cervical dystonia to be 25-33-fold of the previous Botox dosage and the 10-13-fold for the previous Dysport dosage. We report on a 62 year old female patient with antibodies against BTA. For maximum care, the first injection of BTB was performed with the sixfold of the previous Dysport dosage, which was almost the half of the needed dosage predicted from experience with cervical dystonia. The relief only lasted 3 weeks and was therefore disappointing. For the subsequent injections, we consulted Sataloff who also had one patient with antibodies treated with BTB. Based on his personnel advice, we chose the 30-fold dosage, which was effective for 3 months, resulting in improvements to voice quality (both psychoacoustic rating and acoustic measures), voice "quantity" (voice range profiles), aerodynamics (maximum phonation time, phonation quotient), and voice handicap. As with Sataloff et al. (2002), we found that the dosage of BTB probably has to be much higher than in cervical dystonia.

[Do psychological factors change in patients with spasmodic dysphonia after injections with botulinum-toxin?]. / Verändern sich psychologische Faktoren bei Patienten mit spasmodischer Dysphonie nach Botulinum-Toxin-Injektion?

OBJECTIVE: A pre-/posttherapeutic comparative study was carried out to investigate possible associations of voice symptoms, selected psychological factors and their changing. SAMPLE: A total of 11 patients with spasmodic dysphonia (adductor-type) subjected to botulinum-toxin-therapy. Patients had received treatment without any complications for at least 2 years. During that period of time, they received a minimum of 3 and a maximum of 9 injections (mean: 6). METHODS: Psychosocial data were measured before and after treatment using standardized psychological methods. The time span between measurements ranged from 1;8 to 4;6 years, with an average of 2;1 years. RESULTS: On average, no somatic disorder, no clinically meaningful states of anxiety or pathological manifestations of personality traits could be detected, neither pre- nor posttherapeutically. The average stress load caused by life-changing-events was unobtrusive. Before treatment, 5 of 11 patients had an affective disorder in terms of a depressed mood. This caused a higher than average mean depressivity score for the group as a whole. It was no longer found posttherapeutically. CONCLUSION: The depression is interpreted as a psychic reaction. An association between spasmodic voice symptoms and psychosocial genesis could not be proved.

[Bilateral bone anchored hearing aid management of children with external ear dysplasia and/or ear canal atresia]. / Beidseitige BAHA-Versorgung bei Kindern mit Ohrmuscheldysplasie und/oder Gehörgangatresie.

BACKGROUND AND OBJECTIVE: The literature gives hardly any information about the benefits for children of using a bilateral bone-anchored hearing aid (BAHA) as opposed to a bilateral conventional bone conducting hearing aid. PATIENTS AND METHODS: Within a period of 12 years, three children were selected for treatment with bilateral BAHA. At the time of surgery, the children were aged between 8 and 10 years. Two of the children suffered from Franceschetti syndrome and one child from Goldenhar's syndrome. The clinical course of these three patients is presented. RESULTS: In all cases, treatment with BAHA was beneficial according to subjective and objective criteria despite localized inflammatory complications in two of the three cases. CONCLUSIONS: The bilateral use of BAHA should be considered more frequently as an alternative to conventional bone conducting hearing aids even for children.

Progressive sensorineural hearing loss in children with mitochondrial encephalomyopathies.

OBJECTIVE: Mitochondrial disorders are responsible for a variety of neurological syndromes. Specific mitochondrial DNA mutations have been identified recently in some of these rare disorders. Clinical symptoms may occur in different organs to various extent; often they are associated with progressive hearing loss. The aims of this study were to determine incidence, onset, and characteristics of hearing loss in children with mitochondrial encephalomyopathies and to investigate a possible correlation between the degree of hearing loss and neurological symptoms. In addition, we investigated the prognostic value of hearing loss as a predictor of the disease. STUDY DESIGN: From August 1992 to September 1998, 29 patients ranging in age from 5 to 23 years (mean years) were studied. These children were hospitalized for diagnostic purposes in the neuropediatric department. METHODS: The mitochondrial disorder was diagnosed by clinical and laboratory testings, including analysis of the mtDNA. Audiological evaluation consisted of measurements of pure-tone and speech audiometry, tympanometry, and acoustic refle- threshold testing, auditory brainstem response, and evoked as well as distortion-product otoacoustic emissions. RESULTS: A sensorineural hearing loss was identified in 12 children. Three of these were diagnosed as having classic Kearns-Sayre syndrome; five as having multisystem KSS; two as having the syndrome of mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS); one as having KSS-MELAS overlap syndrome; and one as having Friedreich ataxia. Longitudinal testing was performed in seven children, and in all of them a progression of the hearing loss could be demonstrated. Audiological test results in all 12 children suggested cochlear as well as retrocochlear origin of the hearing loss presenting independently from the severity of hearing impairment. No correlation between the characteristics and degrees of hearing loss and the number and severity of clinical neurological symptoms could be found. CONCLUSIONS: The present study demonstrated a high incidence (42%) of sensorineural hearing loss in children with mitochondrial encephalomyopathies. The progressive nature of the hearing impairment was confirmed by a significant correlation between the duration in years and severity of hearing loss in the children. The hearing loss does not have a prognostic value for the progression of the disorder. Based on our findings, we recommend regular audiometric examinations in patients with mitochondrial disorders.

Individual hemispheric asymmetry in vocal fold control of the squirrel monkey.

In ten squirrel monkeys (Saimiri sciureus), electrodes were implanted into the left and right facial motor cortex at sites producing bilateral vocal fold adduction when electrically stimulated. All animals, in addition, had electrodes in the periaqueductal grey of the midbrain (PAG) at sites producing vocalization when electrically stimulated. In eight out of ten animals, motorcortical stimulation during periaqueductally elicited vocalization caused a change in vocal output. This change, in four cases, was more severe with left-sided ipsilateral motor cortex/PAG stimulation than with right-sided ipsilateral stimulation. In the other four cases, right-sided stimulation was more effective than left-sided stimulation. It is concluded that the majority of squirrel monkeys show a hemispheric asymmetry in vocal fold control with left- and right-sided dominance distributed about equally in the population.

Effects on vocal function and incidence of laryngeal disorder when using a laryngeal mask airway in comparison with an endotracheal tube.

A study of 56 patients was undertaken to determine whether there is a difference in the effect of the laryngeal mask airway and the endotracheal tube on the vocal tract after short-duration anaesthesia. All the patients were interviewed pre- and post-operatively. In 43 patients, it was possible to assess the larynx using videoendoscopy and videostrobolaryngoscopy both pre- and post-operatively. Selected acoustic characteristics were investigated in 51 patients pre- and 18-24 h post-operatively. Thirty-four patients that did not have anaesthesia were used as an age-matched control. The interview showed, that patients in the LMA group complained less frequently of having post-operative laryngeal discomfort. The videostrobolaryngoscopy demonstrated minor lesions of the vocal tract in six patients in the ETT group and in one patient in the LMA group. Of the 12 voice variables evaluated, there was no significant difference in any parameter between the two groups. Both groups had a higher fundamental frequency post-operatively.

[A rare case of psychogenic dysarthrophonia or a complex dyskinesia of the pharynx?]. / Ein seltener Fall einer psychogenen Dysarthrophonie oder eine komplexe Dyskinesie des Pharynx?

We present the symptomatology of a complex articulation-related dyskinesia of the pharynx with intermittent functional velopharyngeal insufficiency. It became permanent gradually after a writer's cramp had remitted without therapy. No final nosologic assignment can be made on the basis of the described observations in the individual case. The development of the disease in the 56-year old male patient ended in occupational disability. It suggests manifestation of psychopathological events in the patient's life history of threshold situations by premorbid psychic vulnerability. The possibility of the etiopathologic interaction of emotional and neurological factors cannot be excluded. However, therapy with local botulinum toxin injections proved to be just as unsuccessful as logopedic treatment.

Spasmodic laryngeal dyspnea: a rare manifestation of laryngeal dystonia.

We describe clinical experiences in the management of three patients with laryngopharyngeal dystonia causing severe breathing problems. In contrast to spasmodic dysphonia, which presents with action-induced involuntary spasm of laryngeal muscles during speaking, all three patients showed laryngopharyngeal spasms primarily during respiration. In analogy to spasmodic dysphonia we propose the term spasmodic laryngeal dyspnea for this rare condition. Localized unilateral botulinum toxin injected into the thyroarytenoid muscle and/or ventricular folds reduced the quantity and quality of spasms and led to a pronounced improvement of breathing problems.

Effects of botulinum toxin on vocal tract steadiness in patients with spasmodic dysphonia.

Since laryngeal botulinum toxin (BTX) injections have become the treatment of choice for spasmodic dysphonia, the purpose of this study was to examine its effects on the stability of the upper vocal tract as compared to the effects on glottic stability. Two different acoustic methods were used to analyze voice samples from 16 patients with adductor-type spasmodic dysphonias before and after BTX therapy and from a normal control group. Independent acoustic analyses were used to determine laryngeal and upper vocal tract stability. The results showed significantly higher values for the standard deviation of fundamental frequency (SDF0), reflecting laryngeal instability, for the patient group than for the control group and an impressive improvement for the patients after BTX therapy. Further, the equally high values of SDF0 for the initial second and a second from the midsegment of phonation were differentially reduced by BTX therapy, resulting in a normal pattern of laryngeal stability during sustained phonation. The variability of the first and second formants, reflecting upper vocal tract instability, showed higher values for the patients compared with the control group, but this difference was not statistically significant. The present findings showed that BTX injections to the thyroarytenoid muscle had no discernible effect on stability of the upper vocal tract.

[Functional voice quality assessment in curative microsurgery of laryngeal malignancies. Postoperative voice rehabilitation based on the "laryngeal double valve function"]. / Stimmfunktionelle Qualitätssicherung in der kurativen Mikrochirurgie der Larynxmalignome. Postoperative Stimmrehabilitation auf Basis der "laryngealen Doppelventilfunktion".

According to Negus and Pressman the sphincter systems of the vocal folds and the ventricular folds form a respiratory "laryngeal double valve function". Correspondingly, we found a physiological phonation system of the glottis and a pathological-compensatory one of the supraglottis. They appear to be regulated through an automatic phonatory control system with the glottal phonatory function evidently acting as sensor level. In order to confirm this hypothesis, objective voice analyses with glottal-relevant parameters of 26 voice-rehabilitated patients after minimally invasive laser surgery of glottal carcinomas are presented and integrated into a "hoarseness diagram" with the coordinates roughness and breathiness. Using statistically deliminated acoustic dusters, our data show a qualitative hierarchy of different postoperative phonation mechanisms. They demonstrate the influence of the vibratory capacity of glottal and supraglottal structures on the quality of the vibratory closure. Both functional parameters evidently determine the resulting voice quality in the sense of our hypothesis.

The role of the periaqueductal grey in limbic and neocortical vocal fold control.

The effects of a pharmacological blockade of the periaqueductal grey of the midbrain (PAG) on the elicitability of vocal fold movements from the facial motor cortex on the one hand and the anterior cingulate cortex and dorsomedial hypothalamus on the other were studied in the squirrel monkey. PAG blockade abolished vocal fold activity induced by the cingulate cortex and hypothalamus, but not that induced by the neocortex. These results point to the existence of two separate vocal fold control pathways at midbrain level: one limbic, responsible for non-verbal emotional vocal utterances, and one neocortical, responsible for the production of learned vocal patterns. The PAG represents a crucial relay station of the limbic but not the neocortical vocal control pathway.

[Acoustic voice analysis. On documentation of voice rehabilitation after laser surgery laryngeal carcinoma resection]. / Akustische Stimmanalysen. Zur Dokumentation der Stimmrehabilitation nach laserchirurgischer Larynxkarzinomresektion.

Following curative minimal-invasive laser resection of T1-T3 laryngeal carcinomas, patients were subjected to an intensive voice rehabilitation. Therapy was effected twice a day for approximately 2 months, utilizing the concept of functional voice therapy. Before and after rehabilitation, acoustic analyses were made by using two different computer-supported measuring systems: (1) the "multidimensional voice program" (Kay Elementrics Corp.) and (2) a novel software program developed in Göttingen that includes a new voice quality parameter based on correlations between frequency bands. Acoustic analyses showed superiority of the glottal versus supraglottal compensatory phonation. Findings showed that not all acoustic parameters equally documented voice improvement after rehabilitation. The standard deviation of fundamental frequency was the only parameter showing a significant post-therapeutic improvement. A further suitable acoustic method proved to be the voice quality parameter that has been newly introduced by us. In contrast to analogous parameters of other methods, this approach is independent of the exact periodicity of the glottal excitation function, thus permitting reliable results to be obtained even with aphonic or heavily dysphonic voices.

[Psychological factors in spasmodic dysphonia]. / Psychologische Faktoren bei spasmodischer Dysphonie.

BACKGROUND: Spastic dysphonia has usually been thought as a form of psychogenic dysphonia. A considerable number of authors now feel that this voice disorder is caused by neurological factors. PATIENTS: This study evaluates several psychological factors in 18 patients (9 males, 9 females; mean age: 52.6 years) who had been phoniatrically diagnosed as suffering from adductor spasmodic dysphonia. RESULTS: In standardized psychometric tests, clinical performance of the patients as a group did not deviate from published test norms with respect to emotional instability, hypochondriasis, somatization, or depression. Many patients had been psychosocially stressed to a mild to moderate degree by life events in the two-year period prior to onset of spasmodic dysphonia. These events included severe illness of their own or of near relatives, or death in the family. The personality structure of nearly half of the patients showed a tendency toward increased achievement orientation and certain trait anxieties. CONCLUSIONS: In summary, spasmodic dysphonia remains a phonation disorder of uncertain nosologic classification. It is probable that the disorder is the result of a combination of largely unknown neurological and psychosocial factors.

[Dystonia as the cause of pharyngolaryngeal motility disorders]. / Dystonie als Ursache pharyngolaryngealer Motilitätsstörungen.

Dystonia as cause of pharyngo-laryngeal motility disorders has not been adequately considered in most clinical ENT practices. This case study of a patient with spasmodic torticollis, Meige's syndrome and pharyngo-laryngeal motility disorder was found to be due to dystonia as the underlying cause. The possibility of local symptomatic therapy with botulinum toxin injections has currently provided the physician with an effective means for alleviating the disorder.

Perceptual-acoustic relationships in spasmodic dysphonia.

Perceptual ratings were obtained from voice samples of 19 patients with adductor spasmodic dysphonia before and 1 week after unilateral treatment with Botulinum toxin. Five experienced listeners judged samples of sustained phonation using a seven-point equal-interval scale. The perceptual parameters assessed were overall severity, strain-strangled voice quality, and breathiness. Perceptual results were related to the standard deviation of fundamental frequency and the voice break factor, two acoustic parameters previously shown to be significantly improved following Botulinum toxin injection. Results indicate that in general the spasmodic dysphonia voice is perceived as less severe, less strain-strangled, and more breathy 1 week after treatment. Interrelations among perceptual parameters and relationships with acoustic analyses are discussed.

A comparison of bilateral and unilateral botulinum toxin treatments for spasmodic dysphonia.

To assess the efficacy of bilateral or unilateral botulinum treatments for spasmodic dysphonia we injected botulinum toxin (type A) into the thyroarytenoid muscle of 24 patients with adductor type spasmodic dysphonia. Eleven patients underwent unilateral procedures and 13 bilateral procedures. Samples of sustained phonation were analyzed acoustically by a computer-assisted method and the air flow rates determined. All tests were conducted 1 week before injection and 1 week and 1 month after treatment. With unilateral injection, improvements in acoustic parameters occurred as early as 1 week after treatment. With bilateral injections, only the voice break factor was significantly reduced after 1 week, while standard deviations of fundamental frequency, jitter, shimmer and signal-to-noise-ratios were reduced 1 month after treatment. In comparison with unilateral injections, the mean air flow rate was twice as high 1 week after bilateral injections, with no significant differences found 1 month after treatment. Clinically, both injection modes resulted in the reduction of laryngeal spasms as early as within 48 h after injection.

Vocal tract steadiness: a measure of phonatory and upper airway motor control during phonation in dysarthria.

Acoustic analyses of upper airway and phonatory stability were conducted on samples of sustained phonation to evaluate the relation between laryngeal and articulomotor stability for 31 patients with dysarthria and 12 non-dysarthric control subjects. Significantly higher values were found for the variability in fundamental frequency and format frequency of patients who have Huntington's disease compared with normal subjects and patients with Parkinson's disease. No significant correlations were found between format frequency variability and the variability of the fundamental frequency for any subject group. These findings are discussed as they pertain to the relationship between phonatory and upper airway subsystems and the evaluation of vocal tract motor control impairments in dysarthria.

Effects of botulinum toxin therapy in patients with adductor spasmodic dysphonia: acoustic, aerodynamic, and videoendoscopic findings.

Botulinum toxin has been previously reported to be successful in the treatment of spasmodic dysphonia. To objectively document results, 11 patients with adductor spasmodic dysphonia who received unilateral treatment of the thyroarytenoid muscle were studied. Acoustic analyses and airflow rates during sustained phonation and flexible videoendoscopy were performed prior to, 1 week and 1 month after injection. 1. Acoustic parameters demonstrated significant voice improvement, although abnormal characteristics remained. 2. Mean airflow rates were increased 1 week after injection with almost normal values 1 month later. 3. Videolaryngoscopy showed an effective reduction of intrinsic laryngeal muscle hyperfunction with less effect on extrinsic muscle activity. Interrelations between videolaryngoscopic rating scores, acoustic results and aerodynamic results are discussed.

Phonatory function of neurologically impaired patients.

Previous studies investigated the use of acoustic perturbation measures as a screening tool for neuropathologies with conflicting results. In the present study, five parameters of phonatory function (jitter, shimmer, signal-to-noise ratio, fundamental frequency, and standard deviation of fundamental frequency) were obtained from samples of sustained phonation in three neuropathological groups (Parkinson, Huntington, cerebellar ataxia) and a normal control group to assess the use of acoustic measures in differential diagnosis. In addition, perceptual judgements of the severity of dysphonia for each patient were obtained. The results indicated that perturbation measures of the neuropathological groups showed a higher degree of variability compared to normals. From the five parameters studied, only the standard deviation of fundamental frequency differentiated among neuropathological subgroups. The acoustic parameters studied did not clearly reflect the perceived dysphonia for all subgroups.

[The clinical manifestations of histologically classified malignant lymphomas in the ENT area]. / Klinische Manifestation von histologisch klassifizierten malignen Lymphomen im HNO-Bereich.

For an optimum therapy of malignant lymphomas in the ENT region an extensive analysis of the interrelations of exact histological classification, clinical manifestation and prognosis is desirable. We therefore classified, according to various criteria, 57 patients (34 male, 23 female) aged between 17 and 88, in whom the first diagnosis of a malignant lymphoma was established at our department between 1970 and April 1989. Histologically, in 52 cases (91%) this concerned a non-Hodgkin lymphoma (NHL), in the other 5 cases Hodgkin's disease (HL). According to the "Kiel classification", 60% of the NHL displayed a high degree of malignancy, 36% a low one, while 4% could not be exactly classified histologically. Clinically (Ann Arbor classification), 27 patients with NHL were at stage I (with 21 at stage IE), 16 patients at stage II (with 14 at stage II E), and 9 patients at stage IV. The first manifestation was often extranodular (9 patients tonsil, 8 parotid gland, 8 base of tongue, 7 nasopharynx). A cervical lymph node enlargement was the first sign in 12 patients only. Four patients with NHL additionally developed a second malignancy (squamous cell carcinoma) of another localization. The 5-year survival rate was 81% at stage I, but there were no meaningful differences between stage II (51%) and stage IV (40%). Our study demonstrated that malignant lymphomas of the head and neck are primarily NHL which frequently affect an extranodular organ as a first manifestation. Moreover, malignant lymphomas in the ENT region seem to have a relatively good survival prognosis even in an advanced stage.