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Camurati-Engelmann's disease (progressive diaphyseal dysplasia) is a rare hereditary condition characterized by symmetrical hyperostosis of the long bones and the skull-base, myopathies and neurological disturbances. So far, little more than one hundred cases have been reported. The diagnosis is based on radiological imaging (traditional X rays and CT scan) and family history. Our patient (a 15-year-old male) had high-grade skull-base hyperostosis producing audiological signs, such as progressive deafness, persistent otorrhea and otalgia with progressive stenosis of the external acoustic meatus.

We studied the effect of PTH (10-100 nM) on transductive mechanisms (adenylate cyclase activity, Ca2+ metabolism, IP3 levels) in cell cultures derived from normal and otosclerotic human bone fragments. The cultured cells were osteoblast-like but with calcitonin-receptors still present and with PTH receptors coupled with the adenylate cyclase system. The results showed that PTH activated adenylate cyclase and increased the intracellular Ca2+ levels with qualitative and quantitative differences between the two cellular populations. In particular, otosclerotic cells responded less to hormone stimulation, which is in accord with the current hypothesis of a desensitization of the receptor/enzyme complex associated with the pathological status.

Using an immunoradiometric assay, Cathepsin D (Cath D) levels were measured in the cytosol of 23 normal and 39 neoplastic human laryngeal tissues. Scattered Cath D levels (from 2.2 to 17.8 pM/mg protein; median = 7.6) were found in normal mucosa specimens. Cath D concentrations range from 2.0 to 29.3 pM/mg protein (median = 8.5) in laryngeal tumors. When a comparison between Cath D levels in normal and neoplastic tissue specimens from the same patient was done, Cath D levels were significantly higher in laryngeal cancers than in their normal counterparts (P = 0.03). No correlation with clinico-pathological parameters and steroid hormone and epidermal growth factor receptor status was found. Further studies should investigate whether the production of Cath D by laryngeal tumors could have a clinical relevance for this neoplasia.

Epidermal-growth-factor-receptor(EGFR) expression was evaluated in 103 primary laryngeal tumors and in 42 normal laryngeal tissue specimens. Significantly higher EGFR levels were found in cancer specimens than in normal mucosa (p = 0.0053). EGFR expression did not correlate with age, tumor localization, T classification, cervical-lymph-node involvement or type of surgery, whereas it was higher in poorly differentiated tumors (G3) than in well/moderately differentiated (G1-G2) tumors (p < 0.05). Follow-up data were available for 74 patients. When EGFR status and the most important clinico-pathological characteristics were submitted to univariate analysis, tumor localization, type of surgery and EGFR status were found to be significantly correlated with disease-free survival. The 24-month disease-free survival rate was 58% for EGFR+ cancer patients and 82% for EGFR- ones. With multivariate analysis, only EGFR status and tumor localization were identified as significant independent prognostic parameters. Data reported here suggest that high EGFR levels may identify a sub-set of laryngeal-cancer patients with a particularly unfavorable prognosis.

The authors describe a case of invasive nasosinusal aspergillosis in an immunocompetent patient. After a careful bibliographic review, they emphasize that in the present case the diagnosis was made by means of fungal culture as the clinical picture, imaging techniques and histopathological findings were aspecific. The pathogenetic agent resulted Aspergillus tamarii which belongs to the Aspergillus flavus group and is one of the most unusual agents in literature. The treatment of choice, in the invasive form, is surgery that should be as radical as possible followed by a medical therapy. In the present case, after two-phase radical surgery on all the paranasal sinuses, medical treatment with traconazole was employed with excellent local results at a follow-up of one year.

The authors discuss the epidemiology, histology, symptomatology, diagnosis and therapy of carotid body tumors, basing their discussion on three personal observations and on a case of cervical dermatocyst which showed clinical and radiological data consistent with diagnosis of a glomus tumor. Chemodectomas of the neck are more often seen in the carotid body and are considered histologically benign, although they might induce local or distant metastases. Clinical presentation is often non-specific and may only consist in a slowly growing mass in the higher jugular-carotid region. Among the various imaging techniques (CT, MR), supra-aortic selective angiography, including digital subtraction techniques, is now considered the most reliable tool, while ultrasonography and gammagraphy with 99mTc-gluconate or 131I-MBG are recommended for screening and family testing purposes. Biopsy is to be avoided as it is very likely to cause massive bleeding and because of the high percentage of false negatives. Surgery is the choice treatment, and lateral cervicotomy is the recommended approach for exeresis of the mass, while radiotherapy should be planned in patients with precise contra-indications for surgery and when the tumor mass has already reached the skull base. Pre-operatory selective embolization is still controversial.

Some biosynthetic activities of normal and otosclerotic temporal bone cultures have been studied. Bone cells were cultured for 24 hrs. in medium containing 3H-glucosamine, 35SO4 or 3H-proline. Labelled glycosaminoglycans (GAG) and proteins were precipitated from cells and media. In otosclerotic bone cells there was an evident reduction in the synthesis and secretion of radiolabelled macromolecules. The inhibitory effect was always greater in the extracellular than in the intracellular compartment. Some glycosidases were also studied. Otosclerosis decreased the activity of all enzymes examined, indicating that the lower GAG synthesis and secretion in otosclerotic bone cells were not due to an increased degradation.

The authors describe a rare case of plexiform neurofibroma of the cervical portion of the vagus nerve, and discuss its aetiopathogenesis, clinical, histological and therapeutic features, emphasizing the difference from other benign tumours of the vagus nerve. The clinical characteristics of the mass, ultrasound tomography, CT scanning and digital subtraction angiography were useful in defining its extension and relationships with the surrounding structures. Surgery is the treatment of choice. After mentioning the most commonly employed surgical approaches, they emphasize the advantages of the lateral-cervical approach which allows a wide exposure of the possible sites of origin of the tumour and its complete removal. Finally they stress the need of an accurate histological and immunohistochemical examination in order to differentiate neurofibromas from neurilemmomas.

After outlining the fundamentals of biomagnetism and their possible clinical applications, the authors report the results of a normative study on auditory magnetic fields performed on 18 normally hearing subjects between the ages of 25 and 30. Having presented a thorough review of the literature, they then describe the recording technique employed, the dcSQUID biomagnetic system for signal detection, the shielded room, the characteristics of the stimulus. The auditory magnetic response is characterized by three main waves (P4Om, N100m, P200m) whose latency and amplitude values were calculated. Moreover, in order to localize dipolar activity, certain parameters, such as P and T, were taken into consideration. Localizations were made using a spherical volume conductor or with MRI, which was in any case employed in all the subjects. The waves, especially the N100m recorded contralaterally to the stimulus, showed a reduced latency and an increased amplitude when compared to those recorded ipsilaterally. Moreover, a systematic posterior shift of the N100m source into the left hemisphere with respect to the right one was detected. In conclusion, the authors emphasize the need to study electric as well as magnetic responses in order to better understand auditory cortical functions.

The authors investigated the tubal function by means of sequential scintigraphy in 16 patients affected by chronic otitis media and in 3 patients with posttraumatic perforation of the tympanic membrane. Evaluated parameters were: appearance time (AT), radioactivity peak time, rise time in the tympanic cavity, eustachian tube and rhinopharynx, and radioactivity percent values passed in the rhinopharynx. The tubal pressure-equilibrating function was determined by means of the manometer pump section of the impedance meter. A significant relationship between the pressure-equilibrating function and the tubal scintigraphy parameters has been detected. The variability of the AT in the tube and the almost constant time needed by the radioactive tracer to go through the tube and reach the rhinopharynx could indicate that a major role in the tympanic cavity drainage is played by the tympanic ostium and the surrounding mucosa.

The MLRs of 20 normal hearing adults were recorded by cerebral mapping. In regard to the Pa and Na waves, it is suggested that two different generation sites exist because of the different spatio-temporal distribution of the waves over the scalp. It is demonstrated that amplitudes measured by the "grand average" technique are constantly lower than those obtained with the manual determination, and they conclude that manual determination represents the most reliable method in order to calculate waves amplitude. Finally, it is suggested that, due to the different generation sites for Pa and Na waves and to the uneven distribution of the Na wave over the scalp, Pa amplitude should be measured from the baseline and not from the Na negative deflection. Moreover, in 14 cases, when one ear was stimulated the Pa wave obtained at most recording points was significantly larger than the one recorded contralaterally.

The authors investigated the presence of epidermal growth factor (EGF-R), estrogen (ER), progesterone (PR), and androgen (AR) receptors in 42 laryngeal squamous cell carcinomas and 20 normal laryngeal mucosa specimens. EGF-R were expressed in all tumor specimens analyzed, with significantly higher levels in tumor samples compared with normal mucosa (P less than 0.05). The immunohistochemical study demonstrated the presence of EGF-R in basal and parabasal cells of normal mucosa and in most of the cancer cells. There was no correlation between EGF-R and tumor localization or T classification. On the other hand, the authors did find an interesting correlation between EGF-R levels and grading, EGF-R levels being significantly higher in G3 than in G1-G2 tumors. Moreover, the three patients whose tumors expressed the highest EGF-R levels had disease recur within 8 months. Among tumor specimens, 9.5%, 35.7%, and 9.5% expressed very low but detectable ER, PR, and AR levels, respectively. No statistically significant difference was found between the levels of steroid receptors in the tumor and normal mucosa specimens, and neither was there any correlation of ER, PR, and AR with the pathologic findings. The authors' results suggest that the EGF-R system may play a role in regulating the growth of laryngeal cancer. Additional studies should demonstrate whether, as in other tumor types, EGF-R expression may have prognostic significance in human laryngeal cancer.

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.

The localization and distribution of some cytoskeletal protein components were studied by immunostaining methods in normal and ostosclerotic osteoblast-like cells. The protein components investigated were microtubules (beta-tubulin), intermediate filaments (vimentin), microfilaments (actin and myosin) and structural proteins (alpha-actinin and fibronectin). Although the mechanism is not yet clear, the alterations observed in the pathological cells could well play a role in the expression of otosclerosis.

Steroid hormones are able to influence the metabolism of bone tissue in vivo, but reports regarding their direct action on bone cells fail to agree. In this study, in vitro administration of 17 beta-estradiol, testosterone and corticosterone to normal and otosclerotic osteoblastic cells induced a drop in DNA synthesis in both populations, an increase in the neosynthesis of endocellular proteins in normal cells and a rise, mainly in proteins secreted into the medium, in otosclerotic cells. The fact that 3H-thymidine and 3H-leucine uptake were lower in otosclerotic than in normal cells suggests that the membrane permeability differs in the two populations and that steroids exert an influence on both isotope uptake and directly modulate DNA and protein synthesis.

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.

In order to evaluate the importance of analysis time on posturographic parameters (average X, Y and R, Sway Path, Sway Area, Ellipse Area) both with eyes open and closed, 24 normal subjects were examined for an overall period of 60 seconds. Mean +/- 1 SD values of each parameter were obtained for each time interval (0-20, 20-40, 40-60 sec.) into which the overall period was divided. With the eyes open, the parameters for each time interval did not differ significantly and they were always lower than those obtained with the eyes closed. On the other hand, with the eyes closed, there was a significant reduction in the various parameters as analysis time progressed. In 4 out of 24 subjects an inverse behaviour was detected. The authors conclude that the open-eye analysis time could be limited to 20 sec while, due to changes observed in the various posturographic parameters, closed-eye analysis time should be extended to 60 seconds. The analysis time may prove important in identifying peripheral or central vestibular pathologies since it may be that some of them induce earlier alterations of posturographic parameters while in other pathologies such changes are observed later on.

The authors relate on their own experience concerning etiology, diagnosis and treatment of mandibular fractures. 320 patients (252 males and 68 females) aged between 6 and 82 years with posttraumatic fractures of the mandible, 188 with a single and 132 with multiple fractures, underwent surgical and/or orthopedic treatment. Interosseous wiring and seldom application of metallic plates were the preferred techniques when surgery was performed. Intermaxillary fixation was carried out when the orthopedic treatment was needed. Often both methods were associated. In 10% of the condylar fractures the condyle has been removed. Functional results were excellent and no serious complications could be observed. In 11% of the cases with condylar fractures some limitation of the mandibular movements could be observed, while when condyles were not involved malocclusion could be observed in 3% of the cases. The authors discuss upon etiology, by comparing their own with other experiences, upon diagnosis, which does not seem difficult if adequate radiological investigations are carried out; finally they relate briefly on the advantages and disadvantages of the different surgical and non surgical techniques. Based on their experience they conclude that interosseous wiring and, in selected cases, the application of metallic plates, using an external approach, mostly associated with intermaxillary fixation, represent a safe and adequate treatment for almost every fracture of the mandible.

The authors investigated the 40-Hz steady-state responses (SSR) in 32 full-term newborns and in 10 normal children (5-8 years old), using 500-Hz tone bursts. The 40-Hz SSR threshold is located at about 50 and 30 dB nHL in newborns and older children, respectively. The latencies of both P1 and N1 waves decreased significantly with age, while the amplitudes increased. No significant latency and amplitude intersex differences have been observed. Moreover, with age, the 40-Hz SSR became more stable, their test-retest replicability improved, and P1-N1 wave occurrence increased. The authors finally discuss the possible underlying mechanisms of these findings and conclude that the 40-Hz SSR are difficult to obtain and are scarcely reliable in defining the low-frequency threshold in newborns. The stability and reliability of the responses increase with age, and the electrophysiological and behavioral thresholds to low-frequency stimuli tend to overlap.

74 subjects aged between 60 and 80 years underwent tonal audiometry, speech discrimination tests (phonetically balanced words, normal sentences and compressed sentences) and middle latency responses (MLRs) in order to evaluate possible correlations between behavioural and electrophysiological tests. The results showed that in elderly patients normal sentences are better understood than phonetically balanced words, that performances decrease dramatically when listening conditions became difficult, that auditory threshold seems to influence speech discrimination scores more than does age per se. MLRs seem to be unaffected both by age and by auditory threshold and Pa occurrence is a more reliable parameter than its latency. No correlation between speech discrimination tests and MLRs could be observed; on the contrary in the presence of abnormal speech discrimination tests the MLRs were almost normal and vice versa.

The authors have studied auditory brainstem (ABRs), middle latency (MLRs) and steady-state potentials (SSRs) in 15 adult male rabbits weighing between 2.5 and 3 kg in order to verify if SSRs are due to a mere superimposition of ABRs and MLRs or to a resonance phenomenon. Ten of them were awake while 5 were studied under urethane anesthesia. Acoustic stimuli consisted in 0.1-ms square-wave pulses delivered at presentation rates ranging between 1 and 80/s at a stimulus intensity of 80 dB p.e. SPL. Our data show that reliable auditory SSRs can be obtained in the rabbit at a presentation rate of 30 stimuli/s, probably due to the superimposition of ABRs and MLR Pb waves which show an interwave interval of about 35 ms. The nonlinear aspects which can be detected are probably due to the effect of decreasing interstimulus intervals on the duration and amplitude of the Pb wave. It can then be concluded that SSRs in the rabbit are due more to a superimposition of ABR and MLR waves than to a resonance phenomenon.

The authors answer to the comments on their paper 'Auditory Brainstem Responses to middle-and low-frequency tone pips' by Dr. Paul Kileny. Physical characteristics of the stimuli employed, waveform and latency variations using different high-pass filtering (50 and 200 Hz) are discussed.

16 nasal polyps belonging to 10 male subjects aged between 40 and 70, have been removed during the same number of ethmoidectomies; the polyps have been sectioned and specimens of mucosa corresponding to the pedicle and to the different parts of the body have been collected. During the operation parts of the mucosa of the inferior turbinates were removed. All the specimens have been prepared and observed at the scanning electron microscope. The epithelium of the inferior turbinates, of the small polyps and of the apical part of all polyps were almost normal. Marked alterations of the epithelial layer have been observed in bigger polyps. Surface characteristics of nasal polyps seem to be influenced by their relationship with neighbouring structures and by their position in the nasal cavity, but mostly by their size. On the basis of these findings the authors discuss the most recent etiopathogenetic theories of nasal polyps. They finally describe some peculiar findings concerning the mucosal layer of the polyps and of the inferior turbinates of three confirmed allergic subjects and discuss their possible clinical implications.

Brain stem auditory evoked potentials were recorded from 26 normal subjects who listened to 100 ms clicks at 10, 20, 50, and 100 repetitions per second at varying intensities. A 0.5+/-0.3 ms shift of the V wave latency was observed between 10 clicks per second and 100 clicks per second; a lesser difference was found when the repetition rate was shifted between 10 to 50, 20 to 50, or 20 to 100 clicks per second. No significant differences were observed when the repetition rate moved from 10 to 20 or between 50 and 100 clicks per second. Whatever latency shifts were observed appeared to be independent of the stimulus intensity. Waveform morphology seemed to be the same between 10 and 20 clicks per second, with only minor changes observed at the 50 clicks per second rate. At 100 clicks per second a decrease or a complete disappearance of some components or both was seen. These changes are accounted for by peripheral phenomena for the most part, and a number of clinical applications are suggested based on these observations.

Tests of pulmonary function were performed on children with severe adenoid hypertrophy, before and one month after adenoidectomy. Five types of subjects were selected: (1) normal; (2) cases with isolated increase of residual volume (RV); (3) supernormal type with increased RV; (4) obstructive type of ventilatory defect, and (5) restrictive type of ventilatory defect. Following adenoidectomy there is an objective evidence of improved pulmonary function. The data suggest that 65.7% of clinically normal children with adenoid hypertrophy show pulmonary function abnormalities.

Forty heavy smokers, all males aged between 40 and 70 and affected by laryngeal cancer underwent mucociliary clearance evaluation the day before total laryngectomy, 60 days after and, in 6 of them, 5 years later. Specimens of nasal and tracheal mucosa were obtained during laryngectomy and other subsequent operations. Before total laryngectomy, no significant changes in nasal mucociliary clearance were observed in smokers and controls, and the nasal ciliary carpet was fairly well preserved. Bronchial mucociliary clearance was impaired in all patients, owing to the coexistent chronic obstructive bronchitis. 60 days after the operation, nasal mucociliary clearance was significantly improved when compared with the preoperative data and controls, owing to the increase in the endonasal temperature and humidity, and to the reduction of the nasal blood flow and disappearance of the nasal cycle, which follow tracheostomy. Surface morphologic studies show a change in the squamous epithelium of the anterior third of the nasal fossa into a columnar ciliated one. During the first 3 months after the operation, tracheobronchial mucociliary clearance increased of 50% vis-à-vis the preoperative data. During this period a clinically evident bronchial hypersecretion was observed. The reduction in nasal and tracheobronchial mucociliary clearance function which became evident 6 years after the operation, is probably due to secondary chronic infections.

Fifty-four patients (108 ears) with presumed noise-induced hearing loss, were subjected to tonal and speech audiometry, impedance tests and measurements of auditory brainstem responses (ABR), in order to check for possible retrocochlear involvement. ABR data indicated that latency values of waves I, III and V, as well as III-I, V-III and V-I intervals fell within the normal range in all cases (M +/- 2 SD), even for fast repetition rates (51 stim/s). Poor waveform resolution of early components, particularly of wave I, was found in 12 ears (11.1%) and a total absence of evoked potentials not always related to the hearing loss, occurred in 5 ears (4.6%).

The authors have studied auditory brainstem responses (ABR) in 33 full-term newborns at 0-9, 10-30, 31-58 h, in order to evaluate changes in the first hours after birth, particularly in relation to different repetition rates and recording procedures. The data obtained show that the characteristic newborn 3-peak tracing is observed in almost all the cases at the third recording session while, at the first, wave I is absent in two-thirds of the cases. The most reliable waves at birth are waves III, V and N II, which is often present even when other vertex-positive peaks are absent. Latency values of the considered peaks tend to decrease in a statistically significant way and this decrease is almost complete at the second recording session (30 h), while V-I interval values decrease, especially when the 9 stimuli/s repetition is employed. Stimulus repetition rate affects ABR replicability and synchronization, but it hardly influences latency values. Contralaterally recorded potentials are often absent at birth and become evident 58 h later. The authors conclude that the maturational processes probably affect both peripheral and central auditory structures in the first hours after birth. In this period, increasing repetition rates seem to have a greater effect on wave replicability than on latency values.

In 13 healthy young adults, nasal mucociliary transport velocity was measured by means of albumine microsphere labeled with Tc99m as a tracer. M + ISD of clearance velocity values and of the half time radioactivity (T1/2) resulted to be respectively 1.02 + 0.22 cm/min and 1.07 + 0.24 min. A significant correlation (P less than 0.05) between half time clearance (T1/2) values of the radioactive particles and the mucociliary velocity ones could be detected, making T1/2 a reliable and rapidly obtainable parameter for determining nasal mucociliary function. Advantages and disadvantages of the different techniques are discussed.

40-Hz event-related potentials (AERP) in response to 0.5-, 1-, 2- and 4-kHz tone pips were studied in 45 subjects (18 males and 27 females) in order to assess their reliability and threshold in normal adults and to study the effects of stimulus frequency and intensity on their latency and amplitude. In all subjects well-formed and reproducible 40-Hz AERP were detected, thus showing a good reliability of 40-Hz AERP to tone pips. The response was always detectable within 15 dB nHL intensity level and showed a sequence of positive (P1, P2 and P3) and negative (N1, N2 and N3) waves. It has also been observed that the latency of the first component following the acoustic stimulus decreased at increasing stimulus frequency and intensity, while the amplitude of the whole response increased upon increasing stimulus intensity. It can be suggested that the 40-Hz AERP to tone pips may represent a useful tool in assessing auditory threshold in the low-frequency range.

Pulmonary function of 31 heavy smokers with laryngeal cancer was evaluated before and during the 1st year after total (n = 21) and conservative (n = 10) laryngectomy. 2 of them died because of recurrences, 1 for bronchopulmonary complications. Long-lasting hoarseness was the only presenting symptom in all patients. Preoperative lung function data and mucociliary clearance were consistent with a coexisting chronic obstructive lung disease in most subjects and was probably due to smoking. No differences were observed comparing pre- and postoperative data in the 10 conservative laryngectomy patients. On the contrary, the total-laryngectomy patients showed a progressive impairment of bronchial obstruction and bacteriological infection of the trachea during the 1st year after the operation. An impressive increase in mucociliary clearance rates has been observed 2 months after total laryngectomy during the postoperative hypersecretory phase. the obtained data allow us to hypothesize that when clinical conditions of laryngectomized patients in whom local or distant recurrences have been excluded deteriorate, this is related to a progressive bronchial obstruction at any level of the bronchial tree due to descending bacterial infection of the airways. To our knowledge this is the only work demonstrating that total laryngectomized patients need a complete pre- and postoperative evaluation of lung function, airway dynamics, mucociliary function and tracheal bacteriology for long-term prognosis and treatment.

Thirty-two multiple sclerosis (MS) patients, 10 males and 22 females, aged between 21 and 55, underwent pure-tone audiometry and testing of auditory brainstem response (ABR). Thirteen were classified as 'definite', 12 as 'probable' and 7 as 'possible', according to the McAlpine criteria. Each ear of each patient was tested monoaurally. The most common alterations were seen in the parameters of the cross-correlation between the normal template and the template of the individual MS patient. The second most altered parameters were those concerning the V wave which was absent in most cases but, when present, rarely showed latency-amplitude values falling outside the 90% confidence limit ellipses. Test-retest replicability and stimulations with increasing numbers of stimuli per second were useful in detecting intra-individual variability of waveform characteristics. When considering all the tests performed, 88% of the 'definite', 71% of 'probable' and 64% of 'possible' MS patients' ears showed ABR abnormalities. We stress the importance of a separate evaluation of the two ears due to the frequent unilateral alterations, and of an accurate evaluation of the ABR characteristics. We conclude that brainstem audiometry is a more reliable test for detecting demyelinating processes than is usually described in the literature.

Thirty-four children with clinically and radiologically confirmed adenoid hypertrophy underwent otoscopy, impedance tests, active anterior rhinomanometry and nasal mucociliary clearance evaluation before and 6 months after adenoidectomy. Mucociliary clearance velocity increased significantly while binasal resistances decreased after surgery. The authors conclude that mucociliary evaluation and objective measurements of nasal resistances should be added to impedance tests as indicators to adenoidectomy.

25 subjects aged between 5 and 17 years, 15 females and 10 males, underwent anterior rhinomanometry. 10 of them were normal and 15 affected by velar insufficiency following adenotonsillectomy in 11 case and palatosynthesis in 4. The rhinomanometric tracing was obtained while the patient repeatedly pronounced oral vowels such as a, e, i, c, u and CVs such as ka, ga. In normal subjects intranasal pressure modifications were represented by a series of dyphasic waves with a positive-negative polarity. In the subjects with velar insufficiency the waves were almost monophasic with positive polarity. During and after the phoniatric rehabilitation, waves returned to be dyphasic together with a progressive reduction of hypernasality. While morphology of the rhinomanometric tracing can be considered a reliable index of velar function, wave's amplitude is influenced by the anatomical conditions of the nasal cavity and by the intensity with which the subject pronounces the vowels and the CVs. Rhinomanometry represents therefore an atraumatic, rapid and reliable technique, easy to perform, in order to evaluate velopharyngeal function and to monitor the increase of velar function during and after treatment.

In the present work 30 normal adult subjects (20 males, 10 females; age range 25-40 years) having no signs or symptoms of vestibular involvement were studied in order to verify whether posturographic parameters could be influenced by the static stimulation of cervical and otolithic receptors. The posturographic evaluation was carried out using a stabilometric platform (Bertec) with the subject standing and the head in one of the following positions: median position, turned left (45 degrees), turned right (45 degrees), tilted left (40 degrees), tilted right (40 degrees), with eyes both opened and closed. The data obtained indicate that wide standard deviations exist in the observed parameters and no significant changes were observed with the head turned either to the right or left nor with the head tilted in either direction. Moreover, the posturographic data obtained with eyes open were slightly reduced in comparison to those obtained with the eyes closed. One can, thus, conclude that neither cervical nor otolithic inputs influence posturographic parameters under static conditions, while visual inputs modify significantly posturographic data, except the coordinates of the foot center of pressure.

Mucous membrane's phragments from human normal and neoplastic larynx have been maintained in a organ culture system. A set of cultures has been prepared for optical microscope studies and a parallel set for incorporation studies using 3H Thymidine and 3H Leucine. The results show that there is a good incorporation of 3H Thymidine and a greater incorporation of 3H Leucine at the 7th day due to a differentiation in vitro with a new synthesis of cheratin. These data agree with the morphologic evidence of considerable horny structures in the pathological mucous membrane after 11 days at culture.

The authors have studied calcium 45 uptake and cAMP intracellular levels in normal and otosclerotic bone cell cultures after stimulation with calcitonin in the presence or absence of propranolol. The results seem to demonstrate that poststimulatory 45Ca incorporation is slightly different in normal and otosclerotic cell cultures, being slower but longer lasting in the latter. Propranolol administration markedly inhibits 45Ca uptake in normal cells, while in otosclerotic cells a massive intracellular penetration becomes evident after an initial inhibitory phase. Also cAMP intracellular levels behave differently; a marked increase, followed by a rapid decrease, can be detected in normal cells after stimulation with calcitonin, while in otosclerotic cells, the increase is slower and followed by a long lasting reduction. Adding propranolol reduces cAMP levels in normal cells, while it increases levels in otosclerotic cells. The different behavior of calcium metabolism and cAMP levels after stimulation with calcitonin, depending upon the presence or absence of propranolol, seems to indicate an alteration of the transducing mechanism between stimulus, receptor, and cellular effector in otosclerotic cells.

The effects of sex on I-III, III-V and I-V interwave intervals and I, III, V ABR waves latency values have been studied in 171 normal children born at term, 94 males and 77 females, aged between 2 and 720 days. The obtained data confirm that latency values decrease progressively with age, and show that there is a statistically significant difference in wave III and V latency values and in III-V and I-V intervals between males and females. Moreover, these differences seem to increase with age. The authors also discuss the possible underlying mechanisms and claim that even in infancy, sex-related variability should be taken into account.

The authors report a case of left otorrhagia in a 1-year-old male infant in the presence of a mass involving the parotideal and upper cervical regions, which had appeared after an infection of the upper airways. Non-invasive techniques, such as echography and CT scan, provided useful but contradicting information. Surgery allowed us to define the diagnosis of mycotic aneurysm of the external carotid artery. The authors, after pointing out the extreme rarity of such a pathology, discuss the ethiopathogenetic theories, the clinical features, the diagnosis and the surgical and medical treatment of the disease. Concerning surgery ligation of the external carotid artery is the treatment of choice, since distally the blood flow is provided by a conspicuous collateral circle and because a possible postoperative septic dissemination is avoided.

The authors, after a careful review of the literature during the last 15 years concerning arteriovenous malformation (AVM) of the jaws, report a case of congenital arteriovenous fistula of the mandible characterized by several episodes of spontaneous hemorrhage requiring emergency hemimandibulectomy. Radiologic, arteriographic and pathologic aspects of this lesion are discussed. Even though nomenclature concerning AVM of the jaws is confusing the authors draw attention to the embryologic features which are responsible for these lesions. They also point out the main diagnostic and therapeutic aspects emphasizing the small effect that ligation of the external carotid artery has on bleeding and the importance of choosing case by case the best treatment considering the great variety of clinical and pathologic aspects that characterize these lesions.

The authors first reviewed the main theories concerning the pathogenesis of otosclerosis and studied the morphologic and functional characteristics of cell cultures derived from normal and otosclerotic bones. Light transmission and scanning electron microscopy did not permit definite identification of the cultured cells as predominantly osteoblasts, nor did these techniques show significant differences between cultured cells derived from normal and pathologic bone. Functional tests of the cell cultures proved more interesting. First, the bony nature of the cultured cells was demonstrated by studying the intracellular 45Ca++ uptake after stimulation with calcitonin and dybutryl-cAMP. Second, cell cultures derived from otosclerotic bone behaved differently from those derived from normal bone. Their peak uptake of calcium appeared later, and post-stimulatory values were higher, suggesting that cells derived from otosclerotic bone store a greater quantity of 45Ca++. Furthermore, after stimulation with calcitonin and propranolol, we observed an inhibition of the calcium uptake and decreased intracellular cAMP levels in normal bone cell cultures. In contrast, the cell cultures derived from otosclerotic bone exhibited an initial inhibition of calcium absorption followed by massive calcium penetration. The response of adenylate cyclase to the action of Mg++, Ca++, and F- ions was evaluated in cultures derived from normal bone, otosclerotic bone, and normal skin fibroblasts. The resulting data show that activation due to Mg++ is much lower in cultured cells derived from otosclerotic bone than in those from either normal bone or skin fibroblasts. No significant differences were found after Ca++ inhibition in any of the cell cultures. Moreover, in cell cultures derived from normal bone, F- ions induced a strong activation that was lower than the levels observed in cultures of otosclerotic bone or in normal fibroblasts. We hypothesize that an alteration at the calcitonin receptor site is responsible for the difference in calcium uptake and cAMP levels observed in the cells derived from otosclerotic bone as compared to those cultured from normal cells.

Nasal secretions, maxillary sinus aspirates and specimens of the maxillary sinus mucosa were collected in 44 patients aged between 25 and 60 affected by mono- or bilateral chronic maxillary sinusitis, in order to establish the best sampling technique for microbiological purposes, the most frequently involved bacteria and the physiopathological mechanism underlying chronic maxillary disease. The sinusal mucosa resulted to be the most reliable sample as it reduces contamination and microbial variability. Anaerobic bacteria were isolated in nasal swab (15.6%), in maxillary sinus aspirates (30.4%) and in maxillary sinus mucosa (36.4%) of maxillary sinusitis patients. In controls anaerobic bacteria were isolated only in one nasal swab (2.3%), while they could not be isolated in maxillary sinus aspirates and in maxillary sinus mucosa. The presence of anaerobic bacteria in chronic maxillary sinusitis patients and their absence in controls seem to confirm that anaerobic microorganisms represent the main pathogenetic agents of chronic maxillary sinusitis. The possible physiopathological mechanisms underlying chronic maxillary sinus disease are finally discussed.

ABR (Auditory Brainstem Response) delivering the acoustic stimulus both through a headphone (PHN) and in free field (FF), have been recorded in 59 "normal" children divided into four age-related groups: (1) 22 children born at a gestational age ranging between the 36th and the 41st week; (2) 12 with ages ranging from 1 to 6 months; (3) 15 with ages ranging from 6 to 12 months; (4) 10 with ages ranging from 12 to 36 months. Peak latency values obtained with FF technique have been corrected by calculating the delay due to the distance between the loudspeaker and the tested ears (2.04 ms). When using this correction, no statistically significant differences were found between latency values of peaks JI and JV recorded using the two technique. While JI latency values of various groups do not differ significantly, groups 2--and especially group 1--JV latency values are statistically different from those of the 3rd and 4th group. This observation was confirmed by JV--JI interval values. JV wave in neonates (group 1), using PHN technique, is detectable at 60 dB p.e. SPL (82%) while in all other groups at 60 dB this wave is clearly detectable in all children (100%). Using FF technique, JV wave is still visible at 60 dB in 78% of the neonates (group 1) and in almost all children of the other groups. Considering ABR waveform, using PHN technique, in the first two groups only three waves are visible and the first one disappears at about 80 dB, while the typical 4--5 waves of normal adult tracings are detectable since 8--12 months of age. FF tracings instead show three peaks in all groups, the first (JI) being less evident when compared with the one obtained using PHN technique. JV amplitude values observed in FF are higher than those obtained delivering the stimulus through the headphone.

The authors have investigated the auditory function in 35 Down's subjects, aged between 1 month and 16 years. Clinical examination revealed the occurrence of impaired nasal breathing in 18 subjects (51.4%), while otoscopy results were bilaterally normal in 12 cases (34.3%). Behavioural pure-tone audiometry yielded reliable results in 10 children (28.6%), impedance tests in 28 (80.0%), and brainstem audiometry in 29 (82.9%). A clinical and audiological follow-up has been performed in 11 cases (31.4%). Pure-tone audiometry, which may be employed in all cases only beyond 8 years of age, revealed a conductive hearing loss in 7 cases (20.0%). Impedance tests, whose usefulness is limited by the high occurrence of external ear canal stenosis, showed bilateral type A tympanograms only in 8 cases (28.6%). Stapedial reflex data were often missing, even in presence of a type A tympanogram, due to the weakness of tubaric muscles and to the presence of ossicles abnormalities. ABR has been performed in 29 cases (82.9%) and resulted to be as effective as in the normal population. It revealed a normal configuration concerning threshold and morphology in 16 cases (55.1%). The authors conclude that middle ear pathology in the Down's population is more frequent than expected on clinical basis and that objective tests are mandatory in order to obtain a reliable evaluation. While impedance tests are very sensible in detecting mild middle ear pathologies, but are not effective in threshold definition, brainstem audiometry is the choice tool in the uncooperative child, even if it cannot allow a differential diagnosis between normality and mild low-frequency conductive hearing losses.

Adenylate cyclase (AC) activity was studied in whole homogenates of normal and otosclerotic bone cell cultures. When Mn2+ or Ca2+ was added to the medium there was a similar increase in AC activity in both cell types. F- provoked a greater rise in normal than in pathological cells, whereas 0.01 mM guanosine triphosphate (GTP) significantly raised cAMP synthesis in otosclerotic cells only. Mn2+ + calcitonin (Ct) increased AC activity in both cell preparations. With Ca2+ as cofactor there was no significant rise in either normal or pathological cells. However, while the combination Ca2+ + Ct + GTP had little effect on normal cells, it markedly increased cAMP synthesis in the pathological cells. 1 microgram/ml of the beta-blocker propranolol inhibited the effect Ct exerts on AC in normal cells, but enhanced it in otosclerotic cells. It would, therefore, seem that the pathogenesis of otosclerosis could be associated with an alteration in the AC system associated with Ct receptors.