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S Berrettini

Publications and source records attributed to S Berrettini.

At least 37 records · Page 2Linked to original sources
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 from infectious agents].

The progressive sensorineural hearing loss due to infectious causes can involve different etiological agents like bacteria, viruses, protozoons or mycetes. These infectious agents can act in various ways: directly through a labyrinthitis that may destroy the neuroepithelium; through an ischaemic process secondary to a septic embolus; or through a thrombus. In some cases the damage can occur in a meningitis context, because of the passage of the germ in the inner through the nerves, the vases or the labyrinthine liquids. Bacterial meningitis is one of the causes of progressive sensorinueral hearing loss. Among bacteria, the Mycobacterium Tuberculosis has nowadays acquired a remarkable importance which is also due to its considerable diffusion, despite modern therapy, and to its association with HIV infection. Bacteria can also cause a labyrinthitis acting directly on the inner ear: among these, Treponemas Pallidum, a spirochaete which causes syphilis and Borrelia Burgdorferi, a spirochaete that causes Lyme Disease, must be mentioned. The viruses that are certainly involved in the etiology of progressive sensorineural hearing loss are Cytomegalovirus and Rubella virus. The virus usually causes a labyrinthitis after the viraemia, wich may be due to the passage of the virus from the blood to the endolymph, through the stria vascularis with the consequent infection of the sensorial cells of the organ of Corti. Less frequently the viral damage to the inner ear can occur after a vasculitis, a meningitis or an alteration of the cell-mediated immunity. Progressive sensorineural hearing loss can also occur because of some congenital viral infections such as those caused by Cytomegalovirus and Rubella virus. More recently even the Human Parvovirus B19 seems to have been involved. This virus seems to act through autoimmune and/or immunologic processes, like that causing sudden hearing loss in Lassa fever. Another viral infection which can nowadays more frequently be considered among the cause of progressive hearing loss is HIV. In the HIV infection the neurological toxic lesions due to the administered ototoxic drugs are added up to the damages caused by the opportunistic infectious agents (virus, bacterium, protozoon mycete). However, in these patients HIV itself could be the cause of the auditory and vestibular lesions. More rarely, a progressive hearing loss may be due to the action of a protozoon or mycete only.

Disease Progression↗
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↗
PubMed1997

A case of gustatory rhinorrhoea.

The authors describe a case of gustatory rhinorrhoea that appeared one year after skull trauma with delayed facial palsy. Traumatic interruption and abnormal regrowth of salivary parasympathetic fibers is hypothesized. In order to explain the pathogenesis of this syndrome an anatomical review of the transpetrosal nerves is included. A review of the literature is also presented.

Adult↗
PubMed1996

Acoustic neuroma: correlations between morphology and otoneurological manifestations.

Forty-two patients with acoustic neuroma (AN) were studied to determine whether different types of neuroma could be correlated with specific signs and symptoms of the disease. Based on gadolinium-enhanced TI-weighted MRI sequences, the 42 cases of AN could be divided into three groups, either by size (small: 11.9%, medium: 50%, and large: 38.1%) or by site of origin of the tumour (lateral: 16.7%, intermediate: 69%, and medial: 14.3%). Relations were found between the size and the site of origin of the neuromas and certain clinical, audiological and vestibular findings. The clinical presentation seemed to vary with the site of origin and the size of the tumour: patients with lateral neuromas generally had small tumours, sometimes only located in the internal auditory canal (IAC), and presented early subjective hearing loss while patients with medial neuromas had larger tumours which grew without causing significant audiological symptoms. Normal hearing function was seen only in the patients with medial ANs; however, a significant relation between the size or the site of origin of the AN and the average hearing threshold was not demonstrated. The sensitivity of the stapedial reflex test (SR) was higher for lateral ANs. Anomalies in the brainstem auditory evoked potentials (BAEPs) did not seem to be related to either the size or the site of origin of the AN. The vestibular tests demonstrated a higher frequency of central vestibular involvement in the large tumours, while normal function was more frequent in the lateral tumours. In the group studied the combination of BAEPs and vestibular tests allowed us to identify all the ANs with an optimal level of sensitivity.

Acoustic Impedance Tests↗
PubMed1996

[Allergic fungal sinusitis: is this rare disease an allergy or infection?].

Allergic Fungal Sinusitis (AFS) is a newly recognized form of benign, non invasive sinusitis the histopathologic features of which are similar to those of allergic bronchopulmonary aspergillosis. AFS is a rare condition. However, because treatment and prognosis vary widely, it is important that this disorder be recognized and differentiated from chronic bacterial sinusitis and other forms of fungal sinusitis. AFS does not discriminate by age although it is primarily found in young adults. AFS patients are usually atopic, often having a history of asthma and nasal polyposis. Many have suffered from the symptoms of chronic sinusitis for years while others have had multiple sinus surgery. Radiographs reveal the involvement of multiple sinuses, often with bone destruction. Laboratory findings support an allergic state with a marked increase in eosinophilia and total IgE. At times RAST testing proves positive for fungi and immediate cutaneous reactivity to fungi is also present. Histologic review of the sinus contents reveals characteristic "allergic mucin", with numerous eosinophiles, Charcot-Leyden crystals and fungal hyphae, without any fungi tissue invasion. A wide variety of fungal agents has been implicated, although the majority belong the Dematiacee family. Those patients with allergic mucin but no documented fungi are indicated as having AFS-like syndrome. The pathogenesis of AFS is uncertain. There is controversy in the literature as to what role hypersensitivity (Gell and Coombs type I and type III responses) in infection play. To date current therapeutic recommendations include complete exenteration of all allergic mucin. Adjunctive, short-term systemic steroids often prove useful and nasal steroid spray should be continued for long term. Systemic antifungal agents are not recommended in AFS. Recurrence is common and thus close clinical, endoscopic and radiographic follow-up is important. The clinicopathologic features of one patient with AFS are reported and etiopathogenetic problems are discussed. The presented case showed a positive culture with negative immunological testing (RAST-positive and immediate cutaneous reactivity to fungal antigen), thus confirming the pathogenetic hypothesis of the saprophytic fungal growth in an atopic patient.

Adult↗
PubMed1995

Inner ear involvement in mixed cryoglobulinaemia patients.

In order to evaluate the nature and prevalence of audiovestibular disturbances in mixed cryoglobulinaemia (MC), 32 consecutive MC patients were studied by a wide audiological and vestibular examination. Pure tone audiometry, impedance audiometry, brainstem response audiometry and vestibular function were performed. Patients with a previous history of ear damage due to other well-known agents were excluded from the study. In MC patients we found a rather frequent audiovestibular involvement (34.3%). Bilateral sensorineural hearing loss was found in seven MC patients (22%) and altered vestibular function test values in other seven subjects (22%). Moreover, anamnestic and clinical data revealed a high incidence of benign positional paroxysmal vertigo in our MC series. We can suppose that immune complex-mediated microvascular involvement of the labyrinthine vessels may be responsible for inner ear damage in MC. Thus, audiovestibular disturbances may be included among various organ involvement of the MC.

Adult↗
PubMed1995

[Sequelae of bacterial meningitis in childhood: a study of hearing impairment].

Bacterial meningitis is an infection of the central nervous system involving quite a number of neurological sequelae the most common of which is hearing impairment. To assess the incidence of audiological deficit the authors evaluated retrospectively 20 patients, between 4 months and 11 years of age, observed at the Pediatric Clinic at the University of Pisa between 1988 and 1993. A clinical-neurological examination and a complete auditory assessment (using BAEPs and impedence audiometry) has been performed in every patient between 1 and 18 months after they left hospital. 4 children had persisting neurological sequelae, 3 patients developed sensorineural hearing loss and one child visual impairment and seizures. Haemophilus Influenzae was responsible for 2 cases of sequelae and Streptococcus Pneumoniae for the other 2 cases. The number of days of illness before hospitalization and the institution of an antibacterial treatment, persistence of fever and deviation from the normal level of consciousness and persistence of neck rigidity were not correlated with the presence of sequelae. No correlation has been found between sensorineural hearing loss and the kind of antibacterial therapy. It's advisable that every child, following bacterial meningitis, should undergo a complete and repeated audiological assessment to detect any lesser impairments and/or unilateral losses that may damage the development of speech and language in any way.

Acoustic Impedance Tests↗
PubMed1994

Neuroma of the facial nerve masked by chronic otitis media.

The absence of facial twitching, weakness, or palsy makes the diagnosis of facial neuroma difficult. The authors report a case of neuroma of the horizontal portion of the facial nerve masked by the presence of a chronic ear. A woman with a long history of discharge and hypoacousia in her left ear presented with acute dizziness. Examination revealed grade 3 horizontal right nystagmus, left anacousia, and the appearance of an epitympanic cholesteatoma. Computed tomography (CT) was performed after the vestibular condition improved. The clinical diagnosis of chronic otitis media with cholesteatoma together with the radiologic finding of the mastoid and tympanic cavity completely occupied by soft tissue were enough to send the radiologist astray. The radiologic diagnosis confirmed that the bony destruction of the vestibule and lateral semicircular canal could be caused by a cholesteatoma. A neuroma of the horizontal portion of the facial nerve was discovered during surgery performed for the chronic ear. The postoperative study of the CT scans showed that there was no erosion of the malleus or incus, despite wide erosion of the vestibule and lateral semicircular canal. This finding would be enough to suggest the presence of pathology other than cholesteatoma. The patient refused exeresis of the neuroma. The authors recognize the difficulty in urging a patient to an operation that surely will result in worsening of the facial function. Follow-up in this case has revealed no change in tumor dimension or facial function over 3 years.

Cholesteatoma↗