A retrospective study of audiological function in a group of congenital hypothyroid patients.

A retrospective survey has been carried out on audiological function in 46 congenital hypothyroid cases; mental and physical development were also assessed, as well as the adequacy of substitutive therapy. Using traditional audiometry it was found that 50% of the congenital hypothyroid patients were hypocusic. Severe and profound hearing loss (5 cases) was found in the group of hypothyroid patients with dyshormonogenesis, whereas only one-third of patients with thyroid agenesis presented a mild or moderate hearing loss not related to the time of diagnosis and treatment. The hearing loss was twice as frequent in the patients with clinical hypothyroidism. Using electrophysiological techniques, the early or slow potentials were comparable between hypocusic or normocusic patients; however a longer latency of the slow vertex responses was observed in the hypothyroid group as compared to the normal population. The mean estimated I.Q. was about 2 standard deviations below the average; however 30% of subjects had normal or above normal intellectual level, this percentage rises to 55% if only patients below 16 years are considered. The height was significantly shorter only in the late treated patients (1 year). No correlations were found among audiological and psychological variables, nor between these variables and height. All patients were on desiccated thyroid, but therapy was inadequate in 26% of cases and subclinical hypothyroidism was found in 33% of cases.

IgA transport mechanism through the human nasal mucosa: an immunoenzymatic ultrastrutural study.

The role of secretory IgA antibodies in the antiviral and antibacterial defense of the higher respiratory tract has been widely demonstrated, and the IgA determination in the nasal secretion has become of the highest value in the clinical study. The mechanism by which IgA reaches nasal secretion through the mucosa is still a matter of discussion and neither hypothesis put forward by Tomasi (i.e. free filtration through the epithelic-connectival interstitium or transcellular transport plus active secretion) has so far been fully demonstrated. By using a horseradish peroxidase labeled IgG directed against human IgA we have performed, with suitable controls, a semi-quantitative study on semi-thin and thin section of the IgA behaviour at the level of human nasal mucosa. We have shown that IgA, made up and secreted in the connectival interstitium by specific plasma cells, would be taken up by endocytosis by epithelial cells, where it can be seen especially in the Golgi apparatus and in the large secretion granules. Since in the intracellular transport of pinocytic vesicles actin microfilaments and microtubules undoubtedly play a major role, with ATP utilisation, further studies using either physical factors (e.g. exposure to cold) effecting ATP utilisation or chemical ones (e.g. cytochalasin B and colchicine) depolymering respectively microfilaments and microtubules, should be performed in order to bring further proofs in favor of the active transport IgA mechanism suggested by our present investigations.

Controlled specific immunotherapy of the nasal atopy.

61 extrinsic nasal atopic patients (26 Grass-positive, 35 D.pt.-positive) underwent a longitudinal study of some clinical-diagnostic parameters in order to establish their usefulness for a specific controlled therapy. The symptoms diary drawn up by the patient himself together with the research of the threshold and nasal responsiveness by the RRM provocation test and the evaluation on the same patient of the endo-point of the blocking antibodies result, at present time, the most suitable means to carry out in a satisfactory way a controlled specific therapy.

Eustachian tube permeability during the nasal provocation test.

The results of manometric study on the permeability of the Eustachian tube after a crisis has been produced by exposing 26 cases of perennial nasal atopy to the threshold-dose of the responsible allergen are reported. In 73% of the cases a reduction of the permeability (16 cases) or a stenosis (4 cases) of the ipsilateral tube were found. The importance of inquiring and specifically treating a nasal allergy in patients suffering from secretory otitis is underlined.

Rhino-rheo-manometric (R.R.M.) nasal provocation test.

The nasal provocation test--which has been considered till now too much time-consuming and troublesome for the patient--becomes rapid and simple by the use of the rhino-rheo-manometry. It is possible, by this method, to evaluate the conductance of a single nasal fossa and its reduction after allergen challenge. The results of test performed in patients suffering from extrinsic perennial nasal atopy by Dermatophagoides pt. have proved that the test becomes rapid and not troublesome by using the rhino-rheo-manometry. This method, giving a provocation threshold, may be used both in diagnosis and control of specific immunotherapy.

Secretory immune response after nasal vaccination with live attenuated influenza viruses.

Immunoglobulins and virus-specific antibody in nasal secretions were measured in 18 volunteers before and after intra-nasal vaccination with two live-attenuated type A influenza virus strains. Specific serum antibody rises occurred in 14 out of 18 vaccinees; of those, 11 and 10, respectively, showed significant increase of IgA concentration and of virus-specific antibody in nasal secretions collected 2-4 weeks after vaccination. In one case, a volunteer showed appreciable increase of virus neutralizing antibody in nasal secretions without increase of local IgA concentration and of serum HI antibody. This data indicate that administration of live virus vaccines by upper respiratory route confers effective immunity associated with local antibody synthesis.

Aspirin ototoxicity in the guinea pig.

Aspirin ototoxicity has been studied on guinea pigs by shiver-audiometry and histological investigation of the cochlear duct. One dose of 350 mg/kg has provoked, after 7 h, a mean hearing loss of 18-24 dB at 0.25-8 kHz, followed by complete recovery in 3 days. The difference between the administration of 50 mg/kg/day and 350 mg/kg/day consists of a wide extension of the frequencies involved, and in about 10 dB a greater hearing loss. In both cases, no appreciable recovery of hearing was observed after 22 days (the histological investigations were negative). The biochemical pathogenesis of aspirin ototoxicity is discussed and periodical audiometric controls before and during salicylate treatment are recommended.

Specific local immunotherapy in nasal allergy (preliminary report).

The preliminary results are presented of a study carried out on 23 subjects with nasal allergy to graminacea, 10 of whom were used as a control group to verify the clinical advantages and the possible side effects of a specific local immunotherapy carried out in spray form using progressively increasing doses of allergen in aqueous solution. From a clinical point of view, on the grounds of the score in the clinical diary, we were able to show that during the preseasonal stage only 5 patients treated with the allergen were free of symptoms as were the controls, whereas during the critical phase they were all free of symptoms in contrast to the crisis presented in the control group. The increase in the threshold of nasal provocation and in the serum level of blocking antibodies, revealed in the majority of the subjects affected by pollinosis and treated locally with the allergen, are to be considered fundamental to the clinical advantage obtained. In agreement with what has already been revealed after specific nasal provocation, local treatment with allergens caused in a majority of cases a slight increase in the time of mucociliar clearance, a phenomenon which must be taken carefully into consideration in as much as it could eventually represent an adverse side effect which needs an adequate countermeasure.

Immotile cilia syndrome: radial spokes deficiency in a patient with Kartagener's triad.

Mucociliary transport and ultrastructure of nasal cilia in a 13 year old boy with Kartagener's triad, were investigated. Mucociliary transport was significantly delayed (greater than 30 minutes). Electron microscopy showed cilia lacking radial spokes, eccentric central tubules, and a dislocation of one the outer doublets. Dynein arms were present. We consider the radial spoke defect as a distinct congenital anomaly which contributes to the pathogenesis of the "immotile cilia syndrome".