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.

Sensorineural deafness, hydrocephalus and structural brain abnormalities in two sisters: the Chudley-McCullough syndrome.

We describe an Italian family in which two sisters have macrocephaly due to hydrocephalus, and sensorineural hearing loss in addition to other brain abnormalities demonstrated by Magnetic resonance imaging (MRI). The girls, born to healthy non-consanguineous parents, have borderline psychomotor development delay (probably due to hearing defect) and minor dysmorphisms. The clinical picture fits the Chudley-McCullough syndrome, an autosomal recessive condition, to date described in only five families. Our data, in particular the neuroradiological findings, include all brain anomalies variably reported in previous works (hydrocephalus, corpus callosum partial agenesis, interhemispheric cyst, cerebral and cerebellar cortex dysplasia), thus illustrating the full phenotype of the syndrome.

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 (99m)Tc-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 (EER(10s)). 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.

Acute peripheral vertigo: involvement of the hemostatic system.

Viral infection is the most frequent cause of unilateral acute peripheral vestibulopathy (APV). Another possible cause is a vascular disorder in the labyrinth area associated with alterations in hemostasis. In a group of 45 patients with APV and in a series of 25 patients with Ménière's disease (control group), we evaluated blood parameters, including total cholesterol, triglycerides, apolipoprotein A and B, lipoprotein (a), homocysteine, folate, prothrombin time, activated partial thromboplastin time. D-dimer, fibrinogen, antithrombin III, protein C, protein S, activated protein C resistance, and anticardiolipin antibodies. In the acute phase of their disease, the patients with APV exhibited increased plasma levels of fibrinogen (341.5 +/- 136.8 standard deviation [SD] versus 268.1 +/- 72.6 SD mg/dl; p = .05); increased plasma levels of D-dimer (305 +/- 158 SD versus 201 +/- 106 SD ng/dl; p = .008); enhanced plasma levels of lipoprotein (a) (42.6 +/- 38.5 SD versus 16.9 +/- 17.7 SD mg/dl; F = 5.67, p = .02); high leukocyte count (9.2 +/- 2.7 SD versus 6.4 +/- 1.2 SD x 10(3)/microliter; F = 8.42, p < .006); and low serum folate concentration (5.1 +/- 1.7 SD versus 7.2 +/- 2.6 SD ng/ml; F = 4.34, p = .04). During follow-up, the prothrombin time was prolonged (p = .04), and leukocyte count was decreased (p < .019) in the patients with APV, whereas fibrinogen, D-dimer, lipoprotein (a), and folate were unchanged. In this study, we demonstrated that patients with APV exhibit significant involvement of the hemostatic system.