Biomedical subjects

S Sellari-Franceschini

Publications and source records attributed to S Sellari-Franceschini.

At least 19 recordsLinked to original sources

PubMed2005

Poorly specific binding of thyroglobulin to orbital fibroblasts from patients with Graves' ophthalmopathy.

It has been proposed that thyroglobulin (Tg) may be involved in the pathogenesis or the progression of Graves' ophthalmopathy (GO). According to this hypothesis, following its release from the thyroid, Tg would reach orbital tissues, thereby eliciting an autoimmune aggression. In support of this, we recently found that intact Tg is present in orbital tissues of patients with GO, where it is complexed with glycosaminoglycans. In this study, we searched for additional Tg binding sites in orbital tissues, using primary cultures of orbital and skin fibroblasts from 7 GO patients who had undergone orbital decompression. Biotin-labeled Tg bound to both skin and orbital fibroblasts in a saturable manner, with constants of dissociation of approximately 75 nmol/l for skin fibroblasts and approximately 40 nmol/I for orbital fibroblasts. In an attempt to identify Tg binding sites, fibroblast extracts were blotted onto membranes that were incubated with biotin-labeled Tg, which bound especially to a protein migrating at approximately 300 kDa, present in both orbital and skin fibroblast extracts. Because no appreciable inhibition of binding of biotin-labeled Tg was produced by unlabeled Tg, we concluded that binding was poorly specific and it is unlikely to be involved in the pathogenesis of GO.

Adult↗

PubMed2002

[Intratympanic gentamycin in association with human fibrin glue in the treatment of Meniere's disease: preliminary results].

The treatment of Menierè's disease (Md) with intratympanic gentamicin has rapidly become one of the most widespread alternatives to surgery in this disorder. Numerous studies, employing different protocols, have reported the use of this antibiotic in the treatment of disabling forms of Md, with success rates in the control of vestibular symptoms varying from 73 to 100%, associated with a rate of hearing complications varying from 0 to 75%. We have reported the results of a preliminary experience carried out in 10 patients affected by monolateral Md who were managed with ablation treatment effected with a mixture of gentamicin and human fibrin glue. Upon follow-up examination after one year, all of the patients presented a marked reduction in vestibular reflectivity on the side treated. The vertigo score was zero in all of the patients, showing that the vertigo symptoms were entirely under control. When patients were asked to rate their disability, vertigo was assessed as completely under control in 7 patients and substantially under control in the remaining 3. None of the patients presented any loss of hearing. On the basis of this experience, we propose a standardized protocol which, using an extremely low overall dose of gentamicin, enables elevated success rates to be obtained with the lowest possible number of injections, thus minimizing the risks to hearing. Intratympanic gentamicin associated with a fibrin carrier in Md appears to enable a standardized dose of the drug to be employed, resulting in a decided reduction in the number of administrations and in the overall dose of the drug applied. This makes it possible, with equal benefits in vertigo control, to significantly minimize any hearing loss.

Administration, Topical↗

PubMed2001

The importance of nasal resistance in obstructive sleep apnea syndrome: a study with positional rhinomanometry.

The importance of nasal obstruction in the pathogenesis of obstructive sleep apnea syndrome (OSAS) has not yet been totally defined. Numerous studies have reported an association between nasal obstruction and OSAS, but the precise nature of this relationship remains to be clarified. This study was undertaken to evaluate the prevalence of nasal obstruction disorders in a group of OSAS patients. For this purpose, we analyzed the nasal resistance of 36 OSAS patients by performing a traditional basal anterior active rhinomanometry test (AAR) and a positional AAR, with the patient in a supine position. Seven patients had a pathologic nasal resistance in the seated position that increased further in the supine position; 9 patients had normal resistance in the seated position but a pathologic resistance in the supine position. In 20 patients, nasal resistance was normal in both positions. No statistically significant differences in the degree of apnea/hypopnea index (AHI) was found between the 20 patients with normal positional AAR and the 16 with pathologic positional AAR (p = 0.13). Moreover, no statistically significant differences in the degree of AHI was found between the 7 patients with pathologic basal and positional AAR and the 9 patients with normal basal AAR and pathologic positional AAR (p = 0.38).

Adult↗

PubMed2001

Large vestibular aqueduct in distal renal tubular acidosis. High-resolution MR in three cases.

High-resolution MR of the inner ear performed in 3 consecutive pediatric patients affected by distal renal tubular acidosis (dRTA) and progressive sensorineural hearing loss (SNHL) revealed enlarged vestibular aqueducts (LVA) (bilateral in 1 case and unilateral in 2). LVA is associated to sporadic, progressive SNHL, often secondary to minor head trauma and activities involving the Valsalva maneuver. We believe that the presence of LVA may have contributed to the onset of SNHL and its progression in our patients, and therefore want to stress the importance of morphological studies of the inner ear in patients affected by dRTA and SNHL.

Acidosis, Renal Tubular↗

PubMed2001

Identification of thyroglobulin in orbital tissues of patients with thyroid-associated ophthalmopathy.

Thyroid-associated ophthalmopathy (TAO) is thought to have an autoimmune pathogenesis because of its association with autoimmune thyroid disease, in particular with Graves' disease. Nevertheless, the nature of the autoimmune reaction is unclear, and a target orbital autoantigen has not been conclusively identified. A widely discussed hypothesis is that antigens constitutively shared by the thyroid and orbital tissues are targets of an autoimmune reaction. It has been also postulated that a thyroid-soluble antigen, namely thyroglobulin (Tg), is transported to orbital tissues through the lymphatics, where it accumulates and elicits autoimmune damages in susceptible individuals. Here we have investigated whether Tg is present in orbital tissues from patients with TAO. Retrobulbar tissue specimens were obtained from three patients with Graves' disease and TAO who underwent decompressive orbitotomy, and at autopsy from two patients with no thyroid or eye disease. All patients with TAO had been previously treated with radioiodine to control Graves' hyperthyroidism. Western blot analysis with a monoclonal anti-Tg antibody showed the presence of intact Tg, both in soluble and membrane-associated fractions of orbital tissue extracts from the patients with TAO, in amounts estimated to range from approximately 320 to approximately 900 pg/microg of tissue protein. In contrast, Tg was not detected in orbital tissue extracts from patients with no thyroid or eye disease. Tg was also demonstrated in orbital tissue extracts from two of three patients with TAO by enzyme-linked immunosorbent assay (ELISA), in amounts estimated to range from approximately 450 to approximately 1000 pg/microg of protein. In addition, Tg in orbital tissue extracts from patients with TAO was immunoprecipitated by a rabbit anti-Tg antibody, suggesting that it retained its native conformation. An anti-thyroxine (T4) antibody captured in solid-phase Tg from orbital tissue extracts, showing that it contained thyroid hormone residues and had therefore originated in the thyroid. Tg-anti-Tg immune complexes were not found in orbital tissues, suggesting that if an autoimmune reaction to Tg occurs in TAO, it is likely to be cell mediated.

Aged↗

PubMed2000

High-resolution magnetic resonance and volume rendering of the labyrinth.

Our aim was to verify the feasibility of volume rendering (VR) of high-resolution magnetic resonance (HR-MR) data sets of the labyrinth. We retrospectively reviewed the HR-MR data sets of 16 consecutive patients with no MR evidence of labyrinthine pathology. High-resolution MR data sets were obtained by means of a 3D T2-weighted FSE sequence with the use of a 3-in. circular surface coil for signal reception, and processed with a high-end workstation. Two reviewers performed separately VR of the labyrinth by selecting the signal intensity interval for attribution of opacity and transparency. Concerning the time taken for definition of the volume of interest, the two observers needed, respectively, 28.9 and 33.1 min (SD +/- 8.7-9.5 min), whereas the time taken for VR was respectively, 26 and 33.2 min (SD +/- 8.8-8.9 min). Concerning the selection of the signal intensity interval, the two observers had, respectively, 86.4 and 88.7 mean lower threshold (SD +/- 34.5-33.5), 488.9 and 495.4 mean upper threshold (SD +/- 56.3-53.8). In our experience, we have found VR of HR-MR to offer a reliable and reproducible technique for producing 3D representations of the labyrinth. The VR algorithms use all data within the imaging volume and optimize the dynamic range ascribed to the object being visualized.

Adolescent↗

PubMed1999

Langerhans' cells histiocytosis.

Langerhans cell histiocytosis (LCH) is a rare disorder of unknown cause, characterized by the proliferation of histiocytic cells in various tissues and organs. The role of the otolaryngologist is important in the early and accurate evaluation, staging and diagnosis of LCH, because it may mimic more common diseases such as otitis externa and acute mastoiditis. We discuss a case report of bilateral mastoid involvement in a child with a history of otalgia unresponsive to medical therapy.

Acute Disease↗

PubMed1999

Progressive sensorineural hearing loss in childhood.

A progressive sensorineural hearing loss in childhood, with an extremely variable prevalence (from 4% to 30%), has been reported in the literature. This wide range of reported figures could depend on the different criteria used for identifying the deterioration, the groups, and the examined age ranges. The most frequent etiology of progressive sensorineural hearing loss in childhood includes hereditary causes, both syndromic and nonsyndromic, and developmental and infectious causes, whereas metabolic, toxic, autoimmune, traumatic, and vascular etiologies are less common; however, the origin of the hearing impairment often remains unknown. The population for this study consisted of 178 children with bilateral sensorineural hearing loss who were examined between 1971 and 1993 using audiologic tests. Syndromal genetic hearing loss was excluded from the study. A progressive loss of acuity was found in 11 subjects, with a prevalence of 6.2%. The etiology was hereditary deafness in five patients, congenital infection in one, and congenital inner ear anomaly in another patient; in the last four children the etiology was unknown. Onset of deterioration was after 4 years of age in 73% of the patients. The progressive evolution was binaural in almost all patients (10 of 11) and asymmetric in most, with a tendency to a greater deterioration at the frequencies initially least affected.

Age of Onset↗

PubMed1999

Perennial allergic rhinitis and chronic sinusitis: correlation with rhinologic risk factors.

BACKGROUND: The reported association of allergy and sinusitis varies greatly between study, and the exact role of allergy in predisposing to sinusitis is not clear. We attempted to determine whether patients with perennial allergic rhinitis are at greater risk of developing sinusitis with respect to a control group, and to determine whether there is a correlation between rhinomanometry, endoscopy, and nasal swab, and computed tomography (CT) findings. METHODS: Forty adult patients with perennial allergic rhinitis underwent CT scans of the paranasal sinuses, and the results were then compared to CTs of the paranasal sinuses of 30 control subjects. All allergic patients underwent nasal endoscopy, nasal swab, and active anterior rhinomanometry, and the results were studied in relation to the CT findings. RESULTS: We found sinusitis in 67.5% of the allergic patients and in 33.4% of the controls, with a statistically significant difference between the two groups (P = 0.017). Twenty-three patients had a positive nasal swab; 22 showed increased nasal resistance on rhinomanometry, and 36 had positive endoscopy, but the association of CT findings with endoscopy, rhinomanometry, or nasal swab was not statistically significant (P = 0.583, P = 1.00, P = 0.506, respectively). CONCLUSIONS: Allergic rhinitis is often associated with sinusitis, but the underlying mechanism has yet to be determined. Evidently, factors other than classical pathogen growth and mechanical factors, such as the association of the various factors and immunologic mechanisms, may contribute to the pathogenesis of chronic sinusitis in allergic patients.

Adolescent↗

PubMed1999

[Orbital decompression in Grave's disease: comparison of techniques].

Grave's ophthalmopathy is an inflammatory, autoimmune disorder often associated with Grave's disease. The inflammatory infiltration involves the retrobulbar fatty tissue and the extrinsic eye muscles, causing proptosis, extraocular muscle dysfunction and often diplopia. Orbital decompression is an effective treatment in such cases, particularly when resistant to drugs and external radiation therapy. This work compares the results of orbital decompression performed by removing: a) the medial and lateral walls (Mourits technique) in 10 patients (19 orbits) and b) the medial and lower walls (Walsh-Ogura technique) in 17 patients (31 orbits). The results show that removing the floor of the orbit enables better reduction of proptosis but more easily leads to post-operative diplopia. Thus it proves necessary to combine the two techniques, modifying the surgical approach on a case-by-case basis.

Decompression, Surgical↗

PubMed1998

Progressive sensorineural hearing impairment in systemic vasculitides.

OBJECTIVES: A large series of patients with various forms of systemic vasculitis were evaluated to analyze the prevalence of progressive sensorineural hearing loss (PSNHL), its characteristics and evolution, and the effects of different therapies. METHODS: A total of 673 patients were questioned about the presence of subjective audiovestibular disturbances. Of those, 80 subjects complained of subjective audiological disturbances and underwent oto-rhino-laryngological and audiovestibular evaluation. Those patients with progressive hearing impairment were selected and studied carefully. RESULTS: A PSNHL was observed in 14 patients. The hearing loss was bilateral and asymmetrical in most subjects. It was usually sensorineural, with a cochlear lesion. Unsteadiness was the most frequent vestibular symptom and canal paresis or palsy was noted in most patients. Systemic corticosteroids and cyclophosphamide were useful treatments; in unresponsive patients, satisfactory results were obtained with methotrexate and plasma exchange. CONCLUSIONS: PSNHL is a rare complication of systemic vasculitis, but occasionally is one of the presenting symptoms. Its clinical evolution is variable, but timely clinical assessment and treatment can positively affect prognosis.

Adrenal Cortex Hormones↗

PubMed1998

Herpes zoster oticus: correlations between clinical and MRI findings.

Many gadolinium-enhanced magnetic resonance imaging (MRI) studies focusing on the anatomy and pathology of the 7th cranial nerve have already been published. However, only scattered cases of herpes zoster oticus (HZO) have been described and only the MRI appearance of the soft temporal bone structures has been reported. Enhanced MRI was performed in 4 patients with HZO observed at the Department of Otorhinolaryngology of the University of Pisa. A good correlation was found between the clinical data and MRI findings in both the acute and chronic stages of the disease. The 2 cases with complete facial palsy presented prominent and diffuse enhancement of the 7th and 8th cranial nerves on postcontrast MRI, while the patient with grade III facial palsy showed more limited nerve enhancement. The patient with grade II facial palsy presented no MRI abnormalities. In our series, enhancement limited to the geniculate ganglion and to the labyrinthine segment of the facial nerve indicates a good prognosis while a widespread enhancement correlates with a poor prognosis. In conclusion, MRI with contrast may be useful during the acute stage of HZO because it can confirm the diagnosis and can provide prognostic information on the facial function.

Adult↗

PubMed1998

[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 (Mondini's 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.

Child↗

PubMed1998

[Progressive sensorineural hearing loss: immunologic etiology].

An auto-immune progressive sensorineural hearing loss (PSNHL) can occur as one of the clinical features of systemic immune-mediated disorders, such as Cogan's syndrome, Behçet's disease, Wegener's granulomatosis, mixed cryoglobulinaemia, systemic sclerosis, systemic lupus erythematosus, giant cell arteritis, panarteritis nodosa, relapsing polychondritis, unclassified systemic vasculitides, etc, or as a distinct clinical entity, the so-called auto-immune inner ear disease. The clinical evolution of the hearing loss during the course of the systemic disease is extremely variable (slowly progressive versus rapidly progressive), while the auto-immune inner ear disease is usually characterized by a rapidly developing (weeks or months) progressive hearing loss. In both cases a timely clinical assessment and treatment can positively affect the prognosis of the hypoacusia. To date, no laboratory test will give a sure diagnosis of autoimmune hearing loss and only a good response to corticosteroid and/or cytostatic treatment can indirectly confirm this diagnosis. The laboratory tests that are usually performed can be divided into aspecific and specific tests, the latter evaluating the immune-mediated response to the specific inner ear antigen. Evaluation of the aspecific parameters sometimes shows high values of erythrocyte sedimentation rate and C-reactive protein, but often no alteration of the inflammation parameters is observed. However, the specific tests seem to achieve higher sensibility and sensitivity but to date they are not available in clinical practice. In this study we examined a group of 13 patients affected with bilateral idiopathic PSNHL who underwent Western Blot, which is a specific test for the inner ear antigen. A positive result was found in 53.8% of the cases; in particular, all 3 patients who had an improvement of hearing loss with corticosteroid treatment, and therefore were probably suffering from auto-immune progressive hearing loss, had a positive result to the Western Blot test.

Adult↗

PubMed1998

[Progressive sensorineural hearing loss: metabolic, hormonal and vascular etiology].

Metabolic, hormonal and vascular disorders are considered to cause hearing dysfunction such as progressive sensorineural hearing loss (PSNHL). The diseases most commonly associated with PSNHL are diabetes mellitus, congenital and acquired hypothyroidism, chronic renal failure, chronic labyrinthine ischemia determined by non-hematologic factors (tissue perfusion pressure, blood vessel diameter) or by hematologic factors (blood viscosity and/or rigidity of the red blood cells). In this study a review of data on the relationship between these clinical disorders and the presence and deterioration of the hearing function was carried out. PSNHL seems to be significantly associated with diabetes mellitus, in particular the mitochondrial type: a progressive deterioration of the hearing function is reported in 60% of the patients suffering from this type of disease with mitochondrial mutations. Regarding hypothyroid disorders, the data do not support a pathogenetic link between PSNHL and acquired hypothyroidism, whereas in subjects with congenital hypothyroidism both clinical and experimental results indicate a progressive damage of hearing function if the thyroid disorder is not treated early in life. Also, in patients with chronic renal failure the hearing thresholds seem to be significantly worse than in control subjects, particularly in patients undergoing dialysis, whereas the effect of kidney transplantation on auditory function is still not understood. Finally a relationship between PSNHL and chronic labyrinthine ischemia due to alterations of hematologic factors (increase of blood viscosity and/or increase of rigidity of the red blood cells) has been reported by several authors. However, data on high blood pressure and hyperlipoproteinemia as causes of deterioration of hearing acuity are controversial, and it is often difficult to understand whether the progressive hearing deficit is due only to these factors or also to the elderly age of patients.

Diabetes Mellitus, Type 1↗

PubMed1998

[Progressive sensorineural hearing loss in cochlear otosclerosis].

Otosclerosis is a bone dysplasia limited to the otic capsule causing abnormal resorption and redeposition of bone. The existence of the entity "pure labyrinthine otosclerosis" or "cochlear otosclerosis" is not accepted by all authors; however, there is clinical and histologic evidence to support the existence of a progressive sensorineural hearing loss due to otospongiotic-otosclerotic lesions of the labyrinthine capsule, although diagnosis of this condition may be difficult. The involvement of the inner ear is described as degenerative changes in the spiral ligament, stria vascularis, organ of Corti, and cochlear neurons. The most frequent audiometric configuration is a "bite-type" curve, but flat or rising shapes can also be observed; speech discrimination appears unusually good for a pure sensorineural hearing loss and recruitment is frequently absent. A cochlear otosclerosis should be suspected when there is a family history of otosclerosis, the onset of the hearing loss occurs from the third to fifth decade, and worsening of the hearing loss is observed during periods of intense hormonal and endocrine activity, a positive Schwartze sign is present and bilateral sensorineural loss is associated with signs of unilateral stapedial ankylosis. A definitive diagnosis of cochlear otosclerosis can be made only with computed tomography, which allows a quantitative assessment of the involvement of the labyrinthine capsule by spongiotic or sclerotic areas. The factors to be considered are: otosclerotic foci 1 mm or more in diameter and a density different from that of the normal otic capsule, partially or completely erased contour of the capsule, double ring effect, bony neoformation in the labyrinthine spaces, and increased thickness of the cochlear capsule. The medical management of cochlear otosclerosis is based on sodium fluoride, in association with calcium and vitamin D; some authors have also proposed diphosphonates as inhibitor agents of bone resorption. Surgery may be useful only in those patients presenting a hearing loss so severe that the bone threshold cannot be evaluated and a gap between air and bone conduction cannot be excluded; in these cases stapes operations can improve hearing to a level that may be useful in hearing aid application.

Cochlea↗

PubMed1998

[Diagnostic and therapeutic approach to progressive sensorineural hearing loss].

Progressive sensorineural hearing loss (PSNHL) is an important clinical entity that can develop rapidly and evolve to deafness. The causes of PSNHL can be divided into hereditary, developmental, infectious, autoimmune, metabolic, vascular, neoplastic, toxic and degenerative, even if the origin of the hearing impairment often remains unknown and a diagnosis of idiopathic PSNHL is made. In order to establish the etiology of the hypoacusia or to rule out any known etiology of PSNHL and to diagnose an idiopathic form, a detailed family, physiological and medical history has to be obtained, including history of pregnancies. A careful audiological and otoneurological evaluation should also be carried out, followed by laboratory tests. Furthermore, a complete otoneurological evaluation is required, with puretone audiometry, speech audiometry, impedance audiometry and study of the stapedial reflex, brainstem auditory evoked potentials and vestibular examination. Finally, patients with PSNHL must undergo neuroradiological examination (computerized tomography of petrosal bone, cerebral magnetic resonance and inner ear magnetic resonance); ophthalmological, rheumatological and neuropsychiatric (in childhood) advices should be sought, while other advices may be required on the basis of a particular clinical suspicion. When the etiology is established, therapy must be based on specific treatment for the cause of the hearing deficit; for example, hormonal therapy in alterations of thyroid function, antibiotic treatment in bacterial infections, calcium-antagonist and antiaggregant drugs in the vascular forms, corticosteroids or cyclophosphamide or other immunosuppressive drugs in PSNHL associated with systemic autoimmune diseases. In idiopathic hearing loss, treatment must be started in the absence of important contraindications. The therapeutic protocol that we use in such cases is the following: methylprednisolone boluses, 500 mg/day for three consecutive days, and subsequently oral treatment gradually tapering off in association with a cycle of hyperbaric therapy. Patients showing clinical and historical indications are also treated with intravenous glicerolo, calciparina and/or antiaggregant and calcium-antagonist drugs.

Clinical Protocols↗

PubMed1997

Some uncharacteristic clinical signs and symptoms of acoustic neuroma.

OBJECTIVE: The most common initial symptoms of the acoustic neuroma are unilateral hearing loss that evolves gradually, tinnitus, and unsteadiness. However, atypical presentations may sometimes occur, more often with a small intracanalicular neuroma or with a large medial neuroma placed in the cerebellopontine angle. RESULTS: In our group of 51 patients suffering from acoustic neuroma, atypical presentations were observed in 9 cases (17.6%). Two patients had normal hearing function but reported tinnitus; two patients reported sudden hearing loss, with partial recovery; two patients had a history of fluctuating hearing loss; two patients reported neurologic symptoms (one reported trigeminal paresthesia and the other had a history of trigeminal paresthesia and recurrent headache); and one patient reported profound hearing loss for many years and the recent onset of unsteadiness. CONCLUSION: Patients with these atypical presentations have to undergo a diagnostic evaluation for acoustic neuroma and must be evaluated with BAEPs and then with gadolinium-enhanced MRI.

Adult↗