Can lateral semicircular canal dysplasia play a role in the genesis of hyperacusis.

Hyperacusis can be a prominent and disabling symptom of superior semicircular canal dehiscence associated with autophony and the Tullio phenomenon. We report three clinical cases characterized by disabling hyperacusis in which semicircular canals dehiscence was excluded by temporal bone high-resolution computed tomography. The images disclosed lateral semicircular canal dysplasia, characterized by a small bony island, and dilatation of both the anterior and the posterior arms of the lateral semicircular canal. Cochleo-vestibular examinations (pure tone audiometry, infra-red videonystagmoscopy, vibration-induced nystagmus test, vestibular evoked myogenic potentials) will also be described. To verify the transtympanic ventilation tube effect, bilateral myringotomies tubes were performed in one patient but no long lasting subjective benefit was noted. Concerning the pathophysiology of this condition, we hypothesized that the increased volume of inner ear liquid can modify the micromechanical function of the cochlea and the labyrinthine hydrodynamics. In conclusion, in the case of specific symptoms, such as hyperacusis, it is important to consider the possibility of an inner ear morphological alteration involving the lateral canal and vestibule structures, as well as the existence of bony semicircular canal dehiscence.

Tympanometric findings in superior semicircular canal dehiscence syndrome.

The diagnostic role of audio-impedancemetry in superior semicircular canal dehiscence (SSCD) disease is well known. In particular, since the first reports, the presence of evoked acoustic reflexes has represented a determining instrumental exhibit in differential diagnosis with other middle ear pathologies that are responsible for a mild-low frequencies air-bone gap (ABG). Even though high resolution computed tomography (HRCT) completed by parasagittal reformatted images still represents the diagnostic gold standard, several instrumental tests can support a suspect of labyrinthine capsule dehiscence when "suggestive" symptoms occur. Objective and subjective audiometry often represents the starting point of the diagnostic course aimed at investigating the cause responsible for the so-called "intra-labyrinthine conductive hearing loss". The purpose of this study is to evaluate the role of tympanometry, in particular of the inter-aural asymmetry ratio in peak compliance as a function of different mild-low frequencies ABG on the affected side, in the diagnostic work-up in patients with unilateral SSCD. The working hypothesis is that an increase in admittance of the "inner-middle ear" conduction system due to a "third mobile window" could be detected by tympanometry. A retrospective review of the clinical records of 45 patients with unilateral dehiscence selected from a pool of 140 subjects diagnosed with SSCD at our institution from 2003 to 2011 was performed. Values of ABG amplitude on the dehiscent side and tympanometric measurements of both ears were collected for each patient in the study group (n = 45). An asymmetry between tympanometric peak compliance of the involved side and that of the contralateral side was investigated by calculating the inter-aural difference and the asymmetry ratio of compliance at the eardrum. A statistically significant correlation (p = 0.015 by Fisher's test) between an asymmetry ratio ≥ 14% in favour of the pathologic ear and an ABG > 20 dB nHL on the same side was found. When "evocative" symptoms of SSCD associated with important ABG occur, the inter-aural difference in tympanometric peak compliance at the eardrum in favour of the "suspected" side could suggest an intra-labyrinthine origin for the asymmetry. Tympanometry would thus prove to be a useful instrument in clinical-instrumental diagnosis of SSCD in detection of cases associated with alterations of inner ear impedance.

Otoscopic findings in hypercholesterolemic subjects.

OBJECTIVES: We aimed to verify the extent of a previously cited relationship between tympanosclerosis and atherosclerosis by investigating subjects with dysfunction in lipid metabolism but no clinically apparent symptoms of atherosclerotic disease. METHODOLOGY: Forty hypercholesterolemic patients were submitted to Doppler ultrasound examination of carotid and vertebrobasilar arterial regions; results were matched to otoscopic findings. Otoscopy was performed to evaluate for sclerotic plaques of the tympanic membrane, which represent the most common, clinically non-relevant manifestation of tympanosclerosis. A control group of 41 randomly chosen healthy subjects were also included. RESULTS: Nine (22.5%) of 40 subjects with hypercholesterolemia showed tympanic sclerotic plaques at otoscopy compared to 2 (4.9%) out of 41 control patients. This difference was statistically significant (p = 0.02). An even stronger association (p = 0.01) was found between tympanic and arterial plaques in the study group, as we identified tympanic sclerotic plaques in 7 (41.2%) out of 17 patients with positive Doppler ultrasound signals for arterial plaques. Only two (8.7%) out of 23 subjects without plaques on Doppler ultrasound examination had tympanic sclerotic plaques. CONCLUSIONS: The results of this study confirm the existence of a link between tympanosclerosis and atherosclerosis. To our knowledge, this is the first report of a link between these findings in preclinical atherosclerotic conditions.

Meniere's disease: update of etiopathogenetic theories and proposal of a possible model of explanation.

Meniere's Disease (MD) is an affection consisting of an association of sensorineural hearing loss, tinnitus and vertigo initially presenting by crises. A review of the most considered possible causative factors and pathophysiologic interpretations allows us to underline the uncertainties which still exist about the genesis of this illness. We propose a mechanistic model based on the effect of a haemodynamic imbalance leading to transient ischaemia which could have an effect on the pH of the inner ear as well as on the work of the inner ear proton pumps. It is hypothesized that under ischaemic conditions and consequent metabolic acidity a preserved proton pump activity can generate an overload of anions in the endolymphatic partition, which is a closed system, thus resulting in an enhancement of osmolarity and consequently in the formation of a hydrops resulting in the development of fluctuating hearing loss, tinnitus and vertigo which characterize Meniere's Disease.

Langerhans cell histiocytosis: bilateral temporal bone involvement in an adult with diabetes insipidus.

OBJECTIVE: To present a clinical case of an adult affected by Langerhans cell histiocytosis with bilateral, non-simultaneous, involvement of the temporal bone, associated with diabetes insipidus and to review the literature. METHODOLOGY: A rare case of bilateral temporal bone involvement of Langerhans cell histiocytosis in a 42-year-old woman affected by diabetes insipidus is reported. We present patient's clinical history supported by radiologic, histopathologic and audiologic findings. RESULTS: The patient was submitted to a series of otologic surgical procedures due to the progression of the disease. Ossicular chain was always preserved, so that conservative surgery (canal wall-up technique) was performed, permitting the achievement of good hearing results, bilaterally. CONCLUSIONS: Temporal bone involvement of Langerhans cell histiocytosis may lead to a progressive chronic disease. However, the ossicular chain can remain uninvolved, making a conservative surgical treatment possible. Careful follow-up is essential for detecting new lesions and serial CT scans are mandatory.

Endoscopic treatment of encephaloceles of the lateral wall of the sphenoid sinus.

OBJECTIVE: The suitability of the endoscopic approach for the treatment of an encephalocele of the lateral wall of the sphenoid is discussed. This is a retrospective review of 4 cases diagnosed with temporosphenoidal encephalocele and having a history of CSF leak who were surgically treated using an endoscopic endonasal approach between January 2001 and June 2002 at the Department of Otolaryngology of Sant'Orsola-Malpighi University Hospital and the Department of Neurosurgery at Bellaria Hospital in Bologna. METHODS: Three patients were female between 48 and 73 years of age (mean: 61 years). All patients had suffered from a CSF leak for 5 months to 18 years. None of the patients had a past medical history of head trauma. A fourth patient had undergone a previous microscopic approach for a previously misdiagnosed CSF leak wrongly ascribed to an empty sella. Three patients underwent an ethmoid-pterygo-sphenoidal endoscopic approach (EPSEA), while the patient who had undergone previous microscopic surgery, was treated using a transnasal transsphenoidal endoscopic approach. RESULTS: The follow-up of the patients ranged from 10 to 26 months (mean: 18 months) and no case of a recurrent CSF leak was observed postoperatively. CONCLUSIONS: In our report, the endoscopic approach was a useful tool for the treatment of encephaloceles of the lateral wall of the sphenoid sinus. In skilled hands, this technique permits both the resection of the encephalocele and the subsequent reconstruction of the defect also with a low rate of morbidity.

[Late deterioration in bone conduction after stapes surgery: a retrospective analysis]. / Deterioramento tardivo della conduzione per via ossea dopo chirurgia della staffa: analisi retrospettiva.

In spite of the well-consolidated technique that otosclerosis surgery has built up, there are several aspects that have yet to be satisfactorily explained. One of these is the greater long-term "vulnerability" that appears to characterize an ear that has been operated on compared to a healthy one. In searching for a feasible explanation of this phenomenon and its therapeutic implications, a retrospective analysis of 26 cases was carried out on patients who had been operated between 1966 and 1995 and had come to our attention between 1989 and 1999 due to a rapid, late deterioration in bone conduction. Short-term treatment was pharmacological and surgical in 19 cases and exclusively pharmacological in 7 cases. The analysis of the results of therapy was based on an evaluation criterion of PTA (250-4000 Hz) > 5 dB, calculated on the basis of the bone conduction threshold values. Possible prognostic factors were searched for by means of a multivariate analysis that took as its dependent variable the bone conduction hearing threshold following therapy and as independent variables the age, gender, monolaterality of the otosclerosis ascertained, a positive medical history for analogous phenomena and for previous surgical revision, concomitant vertigo, the time that had elapsed between initial treatment (operation) and deterioration, the technique adopted during the first operation, the extent of the rapid deterioration, the audiometric characteristics at the outset of treatment for the acute episode, the time that had elapsed between deterioration and treatment, the type of treatment, possible reopening of the oval window, and intraoperative findings of a perilymphatic fistula. In the 7 cases managed with pharmacological therapy alone, improvement was seen in 3 cases while the condition remained unvaried in 4 cases; surgical revision (which in 5 cases enabled the presence of a fistula to be ascertained) associated with pharmacological therapy brought improvement in 4 cases, worsening in 4 cases, and no variation of the condition in 11 cases. The only prognostic factor detected, of an unfavourable nature, was the presence of anacusis upon examination. The results obtained do not enable unambiguous conclusions to be drawn: it may in any case be inferred that, at least in certain particular cases, above all when a false cochlear deterioration or the presence of a fistula is suspected, an "aggressive" therapeutic approach may be justified.

Possible role of HMG-CoA reductase inhibitors for the treatment of sudden sensorineural hearing loss (SSHL).

The term 'idiopathic' is used to design sudden sensorineural hearing loss when causative factors cannot be identified. In most cases a viral infection or a circulatory defect are considered to play a significant role when an alternative satisfactory explanation cannot be found. However some significant points remain unclear, since the great variability in age and general conditions of the affected people makes it difficult to find a reliable interpretation of the exact nature of this phenomenon which is reasonably considered to have a multifactorial origin. Under these conditions many different therapeutic strategies have been put forward, thus suggesting some degree of uncertainty not only about the pathogenetic mechanisms of the disease but also on the actual effectiveness of the disparate treatment approaches. In this paper the use of statins in sudden sensorineural hearing loss is firstly proposed on the basis of their metabolic and hemodynamic effects, and the possible clinical implications are outlined.

Systemic hypotension and the development of acute sensorineural hearing loss in young healthy subjects.

BACKGROUND: Sudden sensorineural hearing loss (SSHL) is an acute disorder whose origin is often unclear. A vascular disorder may be a causative factor. OBJECTIVE: To determine whether hypotension influences the genesis of SSHL in healthy subjects. DESIGN: To investigate the role of a 24-hour blood pressure (BP) profile in a population of young subjects with SSHL from January 1, 1996, to December 31, 1999, by a nonrandomized controlled trial. SETTING: The Ear, Nose and Throat Section of the Department of Surgical and Anaesthesiological Sciences and the Department of Internal Medicine, S. Orsola Hospital, University of Bologna, Bologna, Italy. PATIENTS: The study population consisted of 23 untreated healthy patients diagnosed as having SSHL compared with 20 age- and sex-matched normotensive control subjects. Both groups underwent 24-hour BP monitoring, and their BP profiles were analyzed and compared with routine BP values. The data were analyzed with the Statistical Package for the Social Sciences, version 7.1, and the results are expressed as mean +/- SD. MAIN OUTCOME MEASURES: The mean BP values were expected to be lower in the study population. RESULTS: The average clinic and ambulatory BP values were significantly lower in patients with SSHL, for systolic (clinic, P =.004; ambulatory BP, P =.02) and diastolic (clinic, P =.03; ambulatory BP, P =.03) values. The occurrence of persistent hypotension (the presence of >2 consecutive recordings of systolic BP of < or =105 mm Hg and/or diastolic BP of < or =60 mm Hg) was increased in the population with SSHL. CONCLUSION: Systemic hypotension must be considered as the possible cause responsible for the development of SSHL in young healthy subjects.

A relationship between autoimmune thyroiditis and benign paroxysmal positional vertigo?

Benign paroxysmal positional vertigo (BPPV) is a labyrinthine disorder with a typical behavior: intense crises of rotational vertigo induced by postural changes of the head, with short duration and usually good responsiveness to rehabilitative maneuvers. This phenomenon is thought to be subsequent to the movement of floating particles in the labyrinthine fluids, which can provoke gravitational stimulations. In order to conduct a metabolic and autoimmune examination, 70 patients affected by BPPV were examined. In 34 cases (48.5%) autoimmune alterations were found: in 19 cases (27.1%) the level of anti-thyroid antibodies far exceeded the normal values with a significant incidence in comparison with a control group (P<0.01). No other 'risk factors' were present. It can be hypothesized that the diffusion of immune-complexes in the inner ear could change the composition of the endolymphatic fluid exerting a mechanical stimulation of the receptors and provoking the typical vertigo.

Small acoustic neuromas: monitoring the growth rate by MRI.

In a proportion of small acoustic neuroma patients, monitoring with magnetic resonance imaging shows no volumetric increase of tumour size over the years. The object of the study was to identify some indications for the clinical choice between immediate surgery (with the related risks) and watchful waiting. We performed a retrospective study of 47 non-surgically-treated patients affected by acoustic neuroma and monitored by gadolinium-enhanced MRI between January 1990 and February 1999. Six clinical variables (tumour size, sex, age, initial symptoms, ABR pattern and duration of the symptoms) were examined by univariate analysis. Chi-square test and variance analysis were performed to evaluate the statistical significance. In 30/47 (63.8%) cases, no growth was observed during the entire period of follow-up. In the remaining 17/47 (36.2%) patients, a volumetric increase was detected, most often within the first year of observation. The clinical factors examined did not significantly correlate with growth. Despite the relatively short period of observation, we believe that immediate surgery does not need to be considered mandatory for small acoustic neuromas, even in young patients. However the irregular behaviour of the tumour underlines the importance of monitoring with MRI at least once a year.

Hypotension and sensorineural hearing loss: a possible correlation.

A possible role of hypotension in the genesis of sudden or slowly developing sensorineural hearing loss has been outlined. In order to confirm this hypothesis, and to exclude other vascular risk factors, a prospective study was carried out within the "Brisighella Study", a wide and homogeneous group of subjects thoroughly examined from a metabolic and cardiovascular point of view. Among them, 20 participants aged 50 years or less (18 women, 2 men) with diastolic blood pressure < or = 60 mmHg and/or systolic blood pressure < or = 105 mmHg were selected and underwent otological and audiometric examinations. Patients with previous audiological, vestibular and otological diseases were excluded. The control group was represented by 100 subjects (60 women, 40 men), aged 50 years or less, randomly chosen from within a sample of the normal population in the same region. A statistically significant incidence of sensorineural hearing loss was recorded in the study group (7/20 subjects, all affected by low-frequency hearing loss), while hearing impairment was observed in only 3/100 participants in the control group. The mean values of the main metabolic parameters were normal. An alteration of the vasomotor system associated with a hypotensive condition could be responsible as a possible factor in the origin of a cochlear damage and the consequent sensorineural hearing loss.

[Metastasis in the inner auditory canal]. / Metastasi nel condotto uditivo interno.

Metastasis in the inner auditory canal (IAC), or more precisely in the pontocerebellar angle (PCA) is extremely rare. Indeed acoustic neurinoma (AN), meningioma and other types of benign neoformations, in decreasing order, account for nearly all expansive pathologies in this anatomical region. The present paper reports a clinical case of a patient with the sudden onset of an hearing loss in the right ear. This hearing loss was promptly followed by paralysis of the homolateral facial nerve. The function of both cranial nerves worsened progressively and rapidly. Cerebral radiology, performed with MRI and the administration of gadolinium, showed a AN-compatible neoformation fundamentally affecting the IAC. However, histological tests performed after surgery indicated an adenocarcinoma. Post-operative instrumental tests identified the primary neoplasm in the lower lobe of the right lung. The purpose of this work was to highlight, in view of the literature on the topic, the main clinical aspects to consider when a malignant expansion is suspected in the PCA. Naturally there is greater basis for such suspicion when a primary malignant neoplasm has previously been diagnosed. Generally AN progresses quite gradually, in line with an extremely slow growth rate. Therefore it is normally seen in a progressive worsening of hearing, tinnitus and equilibrium disorders. Appearance of a facial nerve palsy is strictly limited to relatively large neoplasms and is encountered at a later stage. This is why a rapid progression of hearing damage accompanied, or promptly followed, by complete facial nerve paralysis must alert one to the possibility of a PCA malignancy. Imaging does not permit easy pre-operative diagnosis since the radiological signs and morphologies are often quite similar to those produced by AN. Therefore, post-operative histological examination can hold some surprises.

Malignant external otitis: review and personal experience.

Malignant external otitis (MEO) is an infrequent but severe infective disorder, generally due to Pseudomonas aeruginosa, which most often affects elderly diabetics patients. The clinical features rarely permit exact diagnosis of MEO to be made promptly, and initially at least they are difficult to distinguish from those of external otitis. This explains the frequent delay in diagnosis with respect to the onset of symptoms. Physical examination almost always reveals the presence of aspecific granulation tissue in the external auditory canal, while the most common laboratory finding is raised erythrocyte sedimentation rate (ESR). Imaging has great diagnostic relevance: CT and MRI are very useful for spatial resolution, while radionuclide scanning and, in our experience, SPECT are superior for detecting early osteitis and monitoring response to therapy. We present an extensive review of the literature and our personal experience. In particular, we stress the relevance of immunological study of MEO patients: all our 4 patients had defective immune defences. As regards therapy, like other authors we underline the fundamental importance of long-term antibiotic treatment. The availability of quinolones and latest generation cephalosporins has greatly simplified the choice of antibiotic treatment, although clinicians should be aware of the possibility of drug-resistant bacterial strains.

[Non-Hodgkin's lymphomas of the salivary gland: analysis of prognostic factors in 28 cases]. / I linfomi non Hodgkin delle ghiandole salivari: analisi dei fattori prognostici su 28 casi.

Primary malignant lymphomas of the major salivary glands are rare and usually arise in the parotid gland (2% of all neoplastic disorders). In this report clinical records of 28 cases of NHL of salivary glands (27 in the parotid gland and one in the submandibular gland) are reviewed and problems related to diagnosis and management strategies are discussed. The 5-year overall survival rate was 72% and did non differ from the survival of other NHL of the head and neck. Statistical evaluation of prognostic factors (age, histology, clinical stage, grading, bulky and surgical approach--biopsy versus parotidectomy), are presented. Analysis of these factors showed that prognosis was not influenced by age, histology, clinical stage and grading of disease. Poor survival was significantly correlated to bulky lesions (tumor size greater than 6 cm). In our experience surgical treatment did not significantly affect survival rate. It is concluded that diagnostic surgical procedures in case of suspected NHL of the parotid gland are fine needle aspiration biopsy. (FNAB) or incisional biopsy. The treatment of choice is radiotherapy associated with chemiotherapy in cases of localized-bulky or disseminated disease.

The problem of the sensorineural component in otosclerotic hearing loss: a comparison between operated and non-operated ears.

The behaviour of bone conduction audiograms in the operated and non-operated ears of 200 otosclerotic patients was analysed. The majority (84%) of both operated and unoperated ears showed virtually unchanged bone conduction thresholds throughout the follow-up period (mean follow-up period = 13.4 +/- 5.3 years). Slight but statistically significant bone conduction deterioration was observed in the remaining 16% of cases, most frequently in the non-operated ears. However, this deterioration was generally within the usually accepted limits of the Carhart effect, and does not demonstrate the presence of any causative factor other than evolution of the ostosclerotic disease. Our findings do not support the hypothesis that total stapedectomy per se may be responsible for sensorineural deterioration.