A specific protocol of myo-functional therapy in children with Down syndrome. A pilot study.

In this article the authors propose a specific myofunctional therapy protocol for children with Down syndrome. For these patients, who usually present with atypical swallowing problems, mouth breathing and lip incompetence, the use of a myofunctional therapy protocol with specific exercises has been shown to improve orofacial and nasal functions. In addition to the functional results, such as the correction of the atypical swallowing, restoration of lip competence, breathing improvement and reduction of nasal rhinorrhea, there were also aesthetic results. This protocol can be useful to improve the quality of life of these patients.

Patients' experiences with HMEs and attachments after total laryngectomy.

OBJECTIVES: The short-term and long-term beneficial effects of HME use by laryngectomees are well described in literature. In this study, we document how laryngectomised patients, who previously did not use an HME, get accustomed to the use of HME and attachments. PARTICIPANTS: Thirty patients, who were at least 3 months post-laryngectomy and previously did not use an HME, were followed for 12 weeks and were asked to complete questionnaires about their experiences with the HME and attachments. RESULTS: Results show that when patients start using an HME, they report some difficulties with breathing resistance during the first 2 weeks of use. However, after 6 weeks, they have become accustomed to the breathing resistance and after 12 weeks over 96% reports that breathing was equal or less strenuous compared with breathing though an open stoma. Only a small proportion of patients experienced problems with increased coughing when starting HME use. CONCLUSIONS: This study provides insight in the way laryngectomised patients are experiencing the use of HMEs in the first weeks. These outcomes can contribute to a better knowledge of HME use by healthcare providers and help them to manage patient expectations and improving support to patients in achieving compliant HME use.

Case report of patients treated with an orthodontic and myofunctional protocol.

BACKGROUND: Occlusion alterations can be associated to bad habits (such as thumb sucking, oral breathing, atypical swallowing and labial interposition) which can lead to functional anomalies. CASE REPORT: Three cases are reported with the good results of myofunctional and orthodontic therapy. CONCLUSION: When there are bad habits, orthodontics should be combined with a myofunctional therapy.

ENT function in a 14-days guinness scuba dive.

Scuba diving is known to affect the rhino-pharyngo-tubaric district (RPT unit). The aim of the study was to document function modifications of the RPT unit in 6 Italian divers (3 men and 3 women) who lived for 14 days consecutively at a depth of 8-10 m, breathing air (21% oxygen) at a pressure ranging between 1.8 and 2 ATA. RPT and inner ear assessment were carried out before the dive (TIME 0) and 24 h (TIME 1) after resurfacing, in order to investigate diving-related RPT and inner ear alterations. Physical examination after resurfacing revealed: fungal external otitis, otoscopic findings consistent with middle ear barotraumas and rhinosinusitis. Rhino-manometry showed a remarkable increase in inspiratory nasal flow and a substantial decrease in nasal resistance. No epithelial cell disruption was retrieved comparing pre and post resurfacing samples. Post-diving tubaric dysfunction was found. Pure tone audiometry revealed a bilateral 40 dB HL hearing loss at 4 kHz in 1 diver. Relevant PTA functions did not seem to be affected by the experiment, no remarkable changes were found at the Sensory Organisation Test and at the Motor Control Test. The 14-day underwater period had a positive effect on nasal flows and resistances.

Patients treated with orthodontic-myofunctional therapeutic protocol.

AIM: The aim of this study is to report three cases that needed myofunctional and orthodontic treatment and the good results achieved after the therapy. Orthodontic treatment alone, in presence of bad habits, is not enough to solve the orthodontic issues, so it needs to be combined with myofunctional treatment.

Causal relationship between malocclusion and oral muscles dysfunction: a model of approach.

AIM: Bad habits result in altered functions which with time can cause anomalies of the orofacial morphology. To solve these problems, orthodontic treatment can be supported by myofunctional therapy in order to recover the normal functionality of the oral muscles. The aim of this study is to assess the need to treat patients with neuromuscular disorders, from both the occlusion and the muscles condition approach in order to obtain the balance needed for the stability of treatment. MATERIALS AND METHODS: A sample of 23 patients with atypical swallowing was included in this study, some of them presented thumb sucking and oral breathing. After case history collection, in order to make a correct orthodontic and functional diagnosis, correction of anomalies was carried out since they could compromise the success of the therapy (maxillary contraction, oral breathing, and short lingual fraenum). Then a different therapeutic approach was applied on the basis of the specific dental features. RESULTS: Both from the diagnostic and therapeutic point of view, important results were achieved especially through muscle analysis with dynamometer and surface electromyography. CONCLUSION: Orthodontic therapy, in the presence of bad habits, is not enough to solve orthodontic issues, it must be combined with a myofunctional treatment. The success of the therapy is granted only when patients and their family comply with the treatment and all factors which can prevent success of the therapy are removed.

Proposal for a myofunctional therapy protocol in case of altered lingual frenulum. A pilot study.

In this article, the authors propose a specific Myofunctional Therapy Protocol for patients with altered lingual frenulum. In such cases tongue muscles are hypofunctioning and their range of motion is reduced. To compensate for this limitation, dysfunctional lingual motor patterns are established, such as the use of some muscles at the expense of others; this negatively affects the development and functions of the stomatognathic system. The Myofunctional Therapy Protocol presented in this pilot study was developed with the aim of making the muscles of the tongue, the floor of the mouth and the soft palate more coordinated, and increasing muscle contraction strength, in order to produce improvements on the muscle tone, on orofacial and nasal functions and a better wound healing and functional recovery in the case of surgical therapy (frenulotomy).

OnabotulinumtoxinA for adductor spasmodic dysphonia (ADSD): Functional results and the role of dosage.

OBJECTIVE: To report the results of functional outcome, dose trend and relationship between onabotulinumtoxinA (onabotA) dosage and the severity of disease or time between therapy sessions in patients affected by adductor spasmodic dysphonia (ADSD). PATIENTS AND METHODS: Thirty-two patients underwent 193 EMG-guided intracordal injections of a starting dose of 2 MU of onabotA. At enrollment, each subject was administered the VHI. The response was evaluated using a subjective rating scale (0-100% of normal phonation). RESULTS: The quality of voice improved significantly after 1 month and stabilized by 3 months. The percentage of normal voice improved 33.34 ±â€¯11.5% (min 26 - max 68). The functional gain was significantly worse in patients presenting with ADSD associated with dystonias in other body regions (31% vs 45% - p < 0.05). The mean dose employed was 3.64 MU (min 1 - max 6) with a trend of increasing dosages up to the 5th treatment after which the doses stabilized over time. The pre-treatment VHI showed a weakly positive correlation with the cumulative dose at the 5th and 10th injections. Benefit duration and the mean between treatment interval were 103 and 136 days respectively. The correlation between dose and inter-injection time is weakly negative (r = -0.22, p < 0.05), however, this is influenced predominantly by the first-to-second injection. After this initial treatment effect, the correlation becomes weakly positive (r = 0.12). CONCLUSIONS: Our data confirm the efficacy of onabotA to improve the quality of voice in cases of ADSD. The trial period for optimal dosage lasted up to a mean of five injections. The dosage of onabotA impacted the length of response and was influenced by the severity of ADSD. Finally the efficacy of onabotA did not change significantly after repeated administrations.

Correlation between musical aptitude and learning foreign languages: an epidemiological study in secondary school Italian students.

SUMMARY: The aim of this study was to assess if a correlation exists between language learning skills and musical aptitude through the analysis of scholarly outcomes concerning the study of foreign languages and music. We enrolled 502 students from a secondary Italian school (10-14 years old), attending both traditional courses (2 hours/week of music classes scheduled) and special courses (six hours). For statistical analysis, we considered grades in English, French and Music. Our results showed a significant correlation between grades in the two foreign languages and in music, both in the traditional courses and in special courses, and better results in French than for special courses. These results are discussed and interpreted through the literature about neuroanatomical and physiological mechanisms of foreign language learning and music perception.

Tensor veli palatini electromyography for monitoring Eustachian tube rehabilitation in otitis media.

BACKGROUND: The pathogenesis of otitis media is related to Eustachian tube dysfunction. The tensor veli palatini muscle actively opens the Eustachian tube and promotes middle-ear ventilation. This study describes a technique for paratubal electromyography that uses a surface, non-invasive electrode able to record tensor veli palatini muscle activity during swallowing. METHODS: Twenty otitis media patients and 10 healthy patients underwent tensor veli palatini electromyography. Activity of this muscle before and after Eustachian tube rehabilitation was also assessed. RESULTS: In 78.5 per cent of patients, the electromyography duration phase and/or amplitude were reduced in the affected side. The muscle action potential was impaired in all patients who underwent Eustachian tube rehabilitation. CONCLUSION: This study confirmed that Eustachian tube muscle dysfunction has a role in otitis media pathogenesis and showed that muscle activity increases after Eustachian tube rehabilitation therapy.

Evaluation of pharyngeal muscle activity through nasopharyngeal surface electromyography in a cohort of dysphagic patients with acute ischaemic stroke.

Oro-pharyngeal dysphagia is frequently present during the acute phase of stroke. The aim of the present study was to evaluate whether the recording of surface EMG using a nasopharyngeal (NP) electrode could be applied to evaluation of pharyngeal muscle activity in acute stroke patients and if this neurophysiological measure is related with clinical assessment of swallowing. Patients were examined and clinical severity was assessed with the National Institute of Health Stroke Scale (NIHSS) score; dysphagia was evaluated through bedside screening test using the Gugging Swallowing Scale (GUSS). Extension of the ischaemic lesion was measured by quantitative score, based on CT scan [Alberta Stroke Programme Early CT Score (ASPECTS)]. We analysed 70 patients; 50 were classified as dysphagic (Dys+), and 20 as non-dysphagic (Dys-). Each participant underwent a surface NP EMG recording performed with a NP electrode, made of a Teflon isolated steel catheter, with a length of 16 cm and a tip diameter of 1.5 mm. The electrode was inserted through the nasal cavity, rotated and positioned approximately 3 mm anteroinferior to the salpingo-palatine fold. At least four consecutive swallowing-induced EMG bursts were recorded and analysed for each participant. Swallowing always induced a repetitive, polyphasic burst of activation of the EMG, lasting around 0.25 to 1 sec, with an amplitude of around 100-600mV. Two parameters of the EMG potentials recorded with the NP electrode were analyzed: duration and amplitude. The duration of the EMG burst was increased in Dys+ patients with a statistically significant difference compared to Dys- patients (p < 0.001). The amplitude was slightly reduced in the Dys+ group, but statistically significant differences were not observed (p = 0,775). Nevertheless, the burst amplitude showed a significant inverse correlation with NIHSS [r(48) = -0.31; p < 0.05] and ASPECTS scores [r(48) = -0.27; p < 0.05], meaning that the burst amplitude progressively reduced with an increase of clinical severity (NIHSS) and topographic extension of brain lesions in CT (ASPECTS). These results suggest that NP recordings can give a semi-quantitative measure of swallowing difficulties originating from pharyngeal dysfunction, in fact, electromyographic findings suggest reduced pharyngeal motility.

Is the identification of the external branch of the superior laryngeal nerve mandatory in thyroid operation? Results of a prospective randomized study.

BACKGROUND: The aim of this study was to investigate the incidence of injury to the external branch of the superior laryngeal nerve (EBSLN) with 2 different surgical approaches. METHODS: From 1998 to 2000, 289 consecutive patients undergoing thyroidectomy were randomly divided into 2 groups. In group A (137 patients [215 upper pole ligations]), the superior thyroid artery was ligated after identification of the EBSLN. In group B (152 patients [244 upper pole ligations]), the superior thyroid artery's branches were ligated separately close to the gland. In all patients, a phoniatric evaluation with videostrobolaryngoscopy and spectrographic examination was performed. RESULTS: The 2 groups were well matched regarding age, sex, thyroid pathological findings, and type of operation. In group A, the EBSLN was not clearly identified in 11.6% of cases. Alterations of EBSLN function were absent in both groups of patients, either postoperatively or 1 and 6 months after operation. Group B showed statistically significant shorter operative time compared with that for group A. CONCLUSIONS: Even if the EBSLN often crosses the superior thyroid pedicle, especially in large goiters, this study demonstrated that accurate distal ligation of the branches of the superior thyroid artery is a safe technique to prevent EBSLN injury.

Overproduction of soluble, extracellular cytotoxin alpha-sarcin in Escherichia coli.

The goal of the present study was to establish the condition to obtain preparative amounts of the recombinant cytotoxin alpha-sarcin to be used for immunoconjugate production. alpha-Sarcin cDNA was isolated from Aspergillus giganteus strain MDH 18,894 and its expression in Escherichia coli was attempted by the use of both two-cistron and fusion protein-expression systems. Whereas the former resulted in low intracellular expression level of recombinant alpha-sarcin (r-Sar), the latter allowed high-level expression of the fusion protein in the culture supernatant. A variant form of alpha-sarcin with an additional threonine residue in position 1 (Thr-Sar) was obtained by proteolytic processing of the fusion protein with a final yield after purification of 40 mg/L of culture. Both recombinant proteins r-Sar and Thr-Sar were identical to native a-sarcin with respect to the biochemical properties and to the in vitro biological activity.

Purification and characterization of two recombinant human granulocyte colony-stimulating factor glycoforms. Pharmacokinetic and activity studies of single-dose administration in mice.

Two recombinant human granulocyte colony-stimulating factor (rhG-CSF) isoforms were isolated from the medium conditioned by an engineered Chinese hamster ovary (CHO) cell line. The two rhG-CSFs were characterized and were found to differ in the carbohydrate structure attached to Thr-133. The glycoform, referred to as Peak 1, contains the O-linked glycan Neu5Ac(alpha 2-3)Gal(beta 1-3)GalNAc; the Peak 2 glycoform contains the O-linked glycan Neu5Ac(alpha 2-3)Gal(beta 1-3)[Neu5Ac(alpha 2-6)]GalNAc. The two glycoforms displayed a similar biological activity in cultures of a mouse 32D C13 cell line and human bone-marrow myelo-monocytic progenitor cells (CFU-GM). In the latter test both glycoforms displayed a higher activity than nonglycosylated rMet-hG-CSF from Escherichia coli. The pharmacokinetic profile and activity of the two rhG-CSF glycoforms and of a mixture of them (Pool) were investigated in mice treated with a single injection of rhG-CSF at the doses of 125 micrograms and 250 micrograms/kg, given via the intravenous (i.v.) and the subcutaneous (s.c.) route, respectively. The plasma concentration profiles obtained were similar for all three substances and did not show any relevant differences in absorption or elimination. The pharmacokinetic parameters indicate that the three substances have similar area under the curve (AUCs), volumes of distribution, and terminal half-life. Furthermore, our data indicate a high bioavailability of the two different glycoforms of rhG-CSF when given to mice via the s.c. route either singularly or as a mixture. Detectable levels of rhG-CSF persisted for more than 8 h in the i.v. and more than 24 h in the s.c. route of administration. All three substances induced early neutrophilia in mice. All rhG-CSF-treated mice developed a two-four-fold rise in neutrophil counts as early as 4 h after the intravenous and 2 h after the subcutaneous injection. Relatively high levels of neutrophils were maintained for at least 8 and 24 h after i.v. and s.c. administration, respectively.

Production and characterisation of a recombinant single-chain anti ErbB2-clavin immunotoxin.

We generated a recombinant immunotoxin, named scFv(MGR6)-Cla, composed of the Fv region of an anti ErbB2 monoclonal antibody (MGR6) fused to clavin, a type 1 ribosome-inactivating protein (RIP) from Aspergillus clavatus. ErbB2 is a tyrosine kinase receptor which is overexpressed in most adenocarcinomas; clavin is a 17 kDa ribonuclease which inhibits protein synthesis by inactivating ribosomes. A recombinant DNA construct containing the cDNA of the single chain Fv fragment (scFv) of the MGR6 antibody fused to the clavin cDNA, was expressed at high levels in Escherichia coli as an insoluble fusion protein containing an N-terminal affinity tag of six consecutive histidine residues. Inclusion bodies were denatured and the recombinant fusion protein was purified under denaturing conditions by single-step purification using immobilised metal ion affinity chromatography (IMAC). The purified immunotoxin was renatured at high yield and histidine tag removed by digestion with enterokinase. The purity of the immunotoxin obtained after refolding was confirmed by SDS-PAGE, RP-HPLC, GPC-HPLC and N-terminal sequence analysis. Cell-free protein synthesis inhibition and binding assays showed that both clavin and scFv(MGR6) maintained their properties after refolding.

Marijuana smoking as a possible cause of tongue carcinoma in young patients.

A case of T1N0M0 carcinoma of the tongue in a male 23-year-old 'regular' marijuana smoker is described. Hemiglossectomy and complete bilateral neck dissection were carried out. No post-operative radiotherapy was given as the resection margins were histologically negative. The tumour recurred one year later in the left cervical region involving the mandible and surgery was again performed, but after three months, neck disease was still evident. The case described case implies the introduction of marijuana as a possible new risk factor in the development of oral cavity tumours. Resection of the primary lesion has to be as wide as possible even in T1 cases, due to the aggressive biological behaviour of such tumours in young subjects.

[Midline granuloma and Wegener's granulomatosis]. / Il "granuloma centro-facciale" e la granulomatosi di Wegener. "Midline granuloma" and Wegener's granulomatosis.

Necrotizing lesions of upper respiratory tract have always been among the most enigmatic diseases of the head and neck region. Nowadays a great deal of nosographic confusion still remains along with numerous doubts concerning diagnostic and therapeutic strategies to be followed in dealing with many diseases often erroneously defined "midline granuloma". In fact, a large variety of diseases appear or may appear as midline destructive lesions in the upper respiratory tract. Each of these, including infections, immune and neoplastic disorders, obviously requires a different therapy. The clinician must have a very good knowledge of the problem in order to make a rational approach to diagnosis and therapy. Wegener's granulomatosis is quite different from "midline granuloma" and must be diagnosed promptly so that an appropriate therapy may be determined as soon as possible. Unlike in the case of "midline granuloma", a prompt therapy is often very effective and gives long periods of remission. In this disease, nevertheless, the etiopathogenesis of both diseases is unknown, precise protocols for diagnosis or treatment do not exist (every case must be considered separately) and prognosis is very poor. On the basis of their personal experience and of an accurate review of Literature, the Authors present a systemic and up-to-date monographic study focusing particular attention on the most recent diagnostic techniques, such as immunohistochemical techniques which utilize monoclonal antibodies, indispensable in the cases of "midline granuloma", and immunofluorescent techniques searching antibodies to cytoplasmic antigens of neutrophil granulocytes, of great value in dealing with Wegener's granulomatosis not only in establishing an initial diagnosis, but also in making prognosis and in controlling the evolution of the disease. The paper also discusses differential diagnosis of midline destructive disorders, highly important for a correct and rational initial approach in diagnosis. Every disease described in the section concerning differential diagnosis must be excluded in order to make an accurate diagnosis of "midline granuloma" in that no typical histopathologic or clinical signs of this particular, destructive disorder. Finally, the authors focus their attention on new etiopathogenetic hypotheses and their therapeutic implications. The most interesting of them are surely those that consider "midline granuloma" the manifestation of a malignant lymphoma as well as those that explain the recent success obtained in treatment of Wegener's granulomatosis using trimethoprim and sulfamethoxazole asserting a possible essential role of infections in the etiology of the disease.

[Validity of schintigraphy in the study of neurogenic dysphagia]. / Validità della metodica scintigrafica nello studio della disfagia neurogena.

The purpose of the present study was to verify the validity and potential application of oropharyngealesophageal scintigraphy in the analysis of neurogenic dysphagia. Scintigraphy was used on 36 patients divided into 2 groups: Group 1 (control) comprised 17 healthy volunteers; Group 2 included 19 patients suffering from various neurological and neuromuscular pathologies (myasthenia gravis, Parkinson's disease, polymyositis, stroke, paralysis of the last cranial nerves). In group 1 scintigraphy provided normal results both for mode of swallowing and transit, and for the values of the various parameters studied. On the other hand, scintigraphy showed that in group 2 all oral, pharyngeal and esophageal phases of swallowing were altered vs the controls with a statistically significant increase in the average values for the oral transit time (OTT) (1.45 sec., p = 0.0005), pharyngeal transit time (OTT) (3.23 sec., p = 0.044), esophageal transit time (ETT) e19.87 sec., p = 0.005) as well as in the corresponding bolus retention indexes ORU (12.95%, p = 0.0003), FIR (15.05%, p = 0.0003) and ERI (28.63%, p = 0.002). Moreover, the quality and means of swallowing also proved altered while tracheobronchial aspiration was only seen in 6 of the 19 patients (maximum value: 90%, average value; 7.66%) with a marked prevalence in the stroke subgroup (4/8). In light of these results and considering the low dose of radiation (0.00043 Gy), the lack of invasiveness and excellent tolerability, scintigraphy has confirmed its clinical validity in the functional, objective and quali-quantitative study of deglutition, even in patients suffering from neurogenic dysphagia.

[Medical-legal evaluation of laryngeal dysfunction to determine disability]. / Valutazione medico-legale delle disfunzioni laringee in ambito di invalidità civile.

The "New Table indicating the percentage of disability resulting from injury and disabling diseases" approved by the Italian Ministerial Decree dated February 5, 1992 gives a superficial, highly disorganized handling of laryngeal pathologies. This Table only considers phonatory function disorders which are included in a special section; it totally neglects dyspnea and dysphagia of laryngeal origin. This omission cannot even be overcome on the basis of similarities since there are no analogous items listed for reference. In fact, the parameters applied to evaluate dyspnea (CV, VEMS, O2 consumption) apply to ventilation disorders of a pulmonary nature and cannot, therefore, be used in cases of laryngeal dyspnea. Moreover, the Table does not deal with dysphagia at all. In addition, the basis for the Table--the concept of permanent functional damage--is not always adhered to in giving indications for evaluation of phonatory tract damage. The purpose of the present work is not, however, to propose a different way to determine the percentages for the individual items in the tables; rather, the aim is to suggest a different, highly standardized procedure for evaluating laryngeal dysfunctions which is easy to apply.

[Ectopic thyroid gland]. / Tiroidi ectopiche.

Based upon the observation of four cases of ectopic lingual thyroid the authors discuss the etiopathogenetic, clinical-diagnostic and therapeutic aspects of this pathology. The clinical characteristics of the mass, scintigraphy, echography and CT scan of the neck, the T3, T4, TSH doses in the blood and fine needle aspiration of the mass have made adequate appraisal of its possible nature and have led to the identification of the presence of thyroid tissue in other sites. In regard to therapy, the authors believe that surgery is compulsory for patients showing clinical signs of upper airway obstruction despite suppressive therapy or when the lesion shows signs of malignant degeneration. After having mentioned the more commonly employed surgical approaches, emphasis is placed on median pharyngotomy as it permits the best visualization of the region, a careful excision of the mass and a valid control over bleeding. In most cases a tracheotomy proves unnecessary. The post surgical hormonal situation suggests the opportunity of a possible substitutive therapy with synthetic hormones or thyroid derivatives.