[Recurrent vestibulo-cochlear pathology due to "immunologic" origin].
Explore the source record for details and available documents.
Adult↗
Biomedical subjects
F Ravecca
Publications and source records attributed to F Ravecca.
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↗
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↗
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↗
[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↗
[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↗
[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↗
[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↗
[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↗
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↗
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↗