Francesco Ursino

Assessment of Swallowing by Oropharyngoesophageal Scintigraphy in Patients with Amyotrophic Lateral Sclerosis

Amyotrophic lateral sclerosis (ALS) is the most common degenerative motor neuron disease in adults, and dysphagia is one of its most frequent and disabling symptoms. Oropharyngoesophageal scintigraphy (OPES) permits a functional and semiquantitative study of the various stages of swallowing. We studied 28 ALS patients (12 females and 16 males; mean age = 63.57 ± 10.39 yr SD), who were clinically rated against the ALSFRS scale (Amyotrophic Lateral Sclerosis Functioning Rating Scale) and underwent OPES with 99mTc-nanocolloid using either a liquid or a semisolid bolus. The semiquantitative parameters we analyzed were Oral Transit Time (OTT), Pharyngeal Transit Time (PTT), Esophageal Transit Time (ETT), Retention Index (RI), and Esophageal Emptying Rate (EER10s). Hence, the OPES performed with a semisolid bolus produced a higher proportion of pathologic values for the swallowing variables than when liquid bolus was used. Analyzed by grouping the patients into classes according to their bulbar ALSFRS scores, we found a significant increase in the OTT (p < 0.005), PTT (p < 0.02), and Oropharyngeal Retention Index (OPRI) (p < 0.0004) variables in ALS patients with more severe bulbar involvement. OPES has turned out to be a very important examination for detecting tracheal-bronchial inhalation and it also offers the possibility of acquiring a semiquantitative evaluation of the amount of food inhaled. In our experience, OPES in patients with ALS has been easy to use, economic, well tolerated, and capable of supplying precise indications with regard to the extent of the swallowing disorder, which permits a better clinical definition of the ALS patient.

Antibodies to inner ear antigens in Meniere's disease

Menieres disease (MD) is an idiopathic inner ear disorder characterized by fluctuating hearing loss, episodic vertigo and tinnitus. Its aetiology is unknown, although there is growing evidence that autoimmunity may be involved in its development. Using the Western blot immunoassay, we examined the reactivity to bovine inner ear antigens of sera from a series of MD patients who had previously been extensively studied for the presence of antibodies to collagens and membrane proteins. Reactivity to inner ear antigens of molecular weight 44 and 53u2003kD was found in 11/25 (44%) and 10/25 (40%) of the patients, respectively; both antigens were absent in the sera of healthy donors. It is still unclear whether the antibodies to 44 and 53u2003kD proteins play a role in the pathogenesis of MD or if they instead represent the result of inflammation and tissue destruction. Even if the latter is true, they may contribute to the perpetuation of the disease or play a role as a cofactor in association with other mechanisms.

Progressive sensorineural hearing loss in childhood

Prevalence of progressive sensorineural hearing loss in childhood seems to be extremely variable, as percentages reported range from 4 to 30%. Differences in the criteria employed for identifying the deterioration, in the groups of patients, and the age range, could explain this wide range of reported figures. The etiology of the progressive sensorineural hearing loss in infants can be hereditary or acquired. Hereditary causes are divided into syndromic and non-syndromic, whereas the acquired causes include congenital or acquired infection (syphilis, cytomegalovirus, rubella virus and toxoplasma infections, bacterial meningitis and acquired viral infections) and congenital inner ear anomalies (Mondinis dysplasia, large vestibular aqueduct, large cochlear aqueduct). Other acquired causes such as disorders of the metabolism, chronic use of ototoxic drugs, autoimmune diseases, perilymphatic fistula and head or acoustic trauma are less common. The age of onset of deterioration shows a great variability because even the congenital hearing losses may occur late after birth. The progressive evolution seems to be binaural in most patients, but more commonly it presents interaural differences, and when the hearing deficit is initially asymmetrical the deterioration is usually greater in the ear which appeared least affected in the first audiogram. Furthermore, at the different frequencies, there is a tendency to a greater deterioration at the frequencies initially least affected, but some authors are not in agreement because they report a uniform pattern of progression in the range of 0.5 to 4 kHz with no modification of the audiometric shape in most of the examined patients.

Ultrastructural and ultracytochemical study of the human nasal respiratory epithelium in vasomotor rhinitis

Objectives—Several pieces of evidence have suggested that nitric oxide (NO) fulfills important functions in the respiratory mucosa, under both normal and pathological conditions. This study was performed to investigate the role of NO in the nasal respiratory epithelium of patients affected by vasomotor rhinitis. The structure and ultrastructure of the epithelium were also examined. Material and Methods—The localization of NO synthase activity was determined by means of reduced nicotinamide adenine dinucleotide phosphate (NADPH)-diaphorase ultracytochemistry. Nasal mucosa was obtained from patients who had undergone surgical therapy for reduction of the inferior turbinate. Results—Examination of hematoxylin–eosin-stained sections revealed that most of the nasal mucosa covering the surgical samples was characterized by severe epithelial damage. The ultrastructural study confirmed the light microscopic observations. Ciliary loss, absence of the intercellular junctions and distension of the intercellular spaces were found in the damaged epithelium. The basement membrane was frequently interrupted. Some epithelial cells were identified as basal cells. Other cells of the damaged epithelium were probably involuted ciliated and goblet cells. The ultracytochemical study showed that the basal cells were NADPH-diaphorase-negative in healthy subjects and strongly NADPH-diaphorase-positive in subjects with vasomotor rhinitis. Conclusions—It is suggested that NO has cytotoxic effects and causes inhibition of mitotic activity in the basal cells, leading to epithelial disruption and breakdown of the protective functions of the epithelium.

Possible association between thyroid autoimmunity and Menière's disease

Various aetiopathological mechanisms have been postulated to be at the root of Menières disease (MD), and some data suggest that there may be also an underlying autoimmune factor. In fact, Menière patients manifest certain characteristics that are typical of autoimmune involvement association of particular human leucocyte antigen haplotypes, the presence of antibodies against internal ear antigens. In this study, we evaluated the association between thyroid autoimmunity and MD in a non‐selected group of patients. We recruited 50 consecutive MD patients and two groups as controls: group A, 82 healthy volunteers; and group B, 50 subjects suffering from acute unilateral peripheral vestibulopathy. All subjects were submitted to instrumental assessment of cochlear–vestibular function and analysis of thyroid‐stimulating hormone (TSH), free triiodothyronine, free thyroxine, anti‐TSH receptor antibody (TR‐Ab), anti‐thyroperoxidase antibody (TPO‐Ab) and anti‐thyroglobulin antibody (Tg‐Ab) in the blood. The prevalence of autoimmune thyroiditis in group B [6/50 (12%); 66·7% TPO‐Ab and 33·3% Tg‐Ab] was superimposable with the healthy controls [6/82 (7%); 66·7% TPO‐Ab and 33·3% Tg‐Ab]. In contrast, 38% of the MD patients (Pu2003= 0·0001 versus group A and group B) had significant autoantibody levels (68·4% TPO‐Ab; 15·8% TPO‐Abu2003+u2003TR‐Ab; 10·5% Tg‐Ab; 5·2% TPO‐Abu2003+u2003Tg‐Ab). Furthermore, 14% of the MD patients were hyperthyroid under l‐thyroxine therapy, while no dysfunction was seen in the control groups. Overall, our data demonstrate a significant association between MD and thyroid autoimmunity, which suggests that an autoimmune factor is involved in the aetiopathogenesis of this disease. These findings suggest that it should be useful to submit MD patients to multi‐disciplinary clinical investigation.

Sex Hormone Receptors in Vocal Fold Tissue: A Theory about the Influence of Sex Hormones in the Larynx

Objective: The larynx is considered a secondary sexual organ. To demonstrate that sex hormones can directly influence laryngeal function, specific receptors in the vocal cord must be identified. Materials and Methods: We searched for estrogen, progesterone and androgen receptors, using an immunohistochemical method, in normal human vocal cords (from 3 cadavers) and in samples of healthy vocal cords and of laryngeal carcinomas from 15 live subjects. Breast and prostate carcinoma were used as controls. Results: In all the normal samples tested, the results were negative; there was only a nonspecific cytoplasmatic response in the subepithelial glands (false positives). In the neoplastic tissue, 2 samples had a weak nuclear focal positivity for estrogen and progesterone receptors; all 15 subjects studied were negative for androgen receptors. Conclusions: Since our data show that sex hormone receptors are absent in the vocal cords, other theories must be considered to explain the fact that hormones influence the quality of the voice. This study discusses the possibility that the changes of voice according to gender and throughout life might be linked with a different expression of some growth factors in the laryngeal tissue and that this expression might in turn be influenced by hormonal variations.

Elevated Antithyroid Peroxidase and Antinuclear Autoantibody Titers in Ménière’s Disease Patients: More than a Chance Association?

Objective/Hypothesis: The aim of this prospective study is to evaluate the possible association between Ménière’s disease (MD) and autoantibodies. Methods: Fifty-five patients with definite MD (51 unilateral and 4 bilateral) were matched with 55 patients with unilateral vestibular paresis without cochlear involvement and 55 healthy subjects. Blood samples were collected from all study subjects for the determination of serum TSH, free triiodothyronine, free thyroxine, anti-TSH receptor antibody, antithyroperoxidase antibody, antithyroglobulin antibody and of antibodies to non-organ-specific antigens, namely antinuclear antibodies, antibodies to extractable nuclear antigens and antineutrophilic cytoplasmic antibodies. Results: Thirty-three subjects (60%) of the MD group had 1 or more elevated serum autoantibody levels, both organ and non-organ specific; 16 patients (29.1%) with unilateral vestibular paresis had 1 or more elevated serum autoantibody levels, while 13 healthy subjects (23.6%) had 1 or more elevated serum autoantibody levels. Conclusions: Based on our data we speculate that there is a more than a chance association between MD and ‘autoimmunity’, thus suggesting a hypothetical role of the immune system in MD pathogenesis. In other words, a pathogenetic role of an ‘immune dysregulation’ in MD patients can be hypothesized.

Neuro-laryngeal involvement in Churg-Strauss syndrome

Abstract We report our clinical experience in managing a 59-year-old Italian male with Churg-Strauss syndrome (CSS) whose first clinical manifestation was a persistent dysphonia; the patient worked as a mechanic. Video-laryngostroboscopic examination revealed paresis of the right vocal fold with a reduction in adduction together with incomplete glottal closure. Spectrographic and spirometric tests both showed abnormal changes. Laryngeal electromyography revealed neurogenic damage of the right thyroarytenoid and crycoarytenoid muscles. Due to the appearance of typical signs of systemic involvement of CSS as a necrotizing vasculitis, the patient was admitted to the Rheumatology Unit of the University of Pisa. Histologic analysis of a skin lesion on the patient’s foot confirmed the diagnosis. Treatment with 6-methylprednisolone quickly brought remission from systemic and laryngeal symptoms, as well as improvement in the results of video-laryngostroboscopic, spectrographic and laryngeal myographic tests.

Distribution of 3-nitrotyrosine in the nasal polyps of atopic patients.

Objective To investigate whether formation of nitrotyrosine in the nasal polyps of atopic patients occurs.

Respiratory Retraining Therapy in Long-Term Treatment of Paradoxical Vocal Fold Dysfunction

Objectives: Paradoxical vocal fold dysfunction (PVFD) is a disorder in the larynx featuring involuntary adduction of the vocal folds during the inspiratory phase of breathing. The symptoms include acute episodes of dyspnea and bouts of coughing. To date, there is no universally acknowledged treatment for PVFD, though respiratory retraining therapy is the treatment of choice. Aims: The purpose of this work was to evaluate the results of long-term respiratory retraining therapy in cases of PVFD. Patients and Methods: We treated 20 patients with PVFD for 2 years: 10 subjects were submitted to a cycle of respiratory retraining therapy every 12 months (receiving a total of 3 cycles) while 10 were given a cycle every 3 months (for a total of 9 cycles) no matter what their clinical conditions were. Results: The results show that long-term respiratory retraining is particularly efficacious if the cycles of treatment are repeated, no matter what clinical conditions are present. In fact, when only one cycle of retraining treatment is given a year, there is initial improvement followed by progressive worsening. Conclusions: Long-term respiratory rehabilitation is effective, especially if the treatment is given at least once every 3 months.