Early management of hearing and balance disorders: a review of literature and a proposal to overcome possible uncertainties.

The lack of a correct diagnostic and therapeutic planning of vestibular diseases is still often observed, and some difficulties are still to deal with in clinical practice, even when treating acute hearing problems, although the needed competence is more easily identified as otologic. A review of the international literature confirms the existence of such a problem, and permits to underline the scarcity of connections between otology and neurotology, on one hand, and principles of basic sciences and general and internal medicine, on the other hand: this can explain some therapeutic contradictions in treating inner ear disorders, their frequent labelling as idiopathic and the persisting uncertainties concerning a correct diagnostic and therapeutic management. In order to overcome the difficulties deriving to insufficient interdisciplinary cooperation, the institution of hospital audiovestibular services with a strictly linked net of cooperation with internal medicine units could represent a progress. This could help prevent clinically and economically inadequate management, contributing to minimize the possibility of expensive and/or health-threatening mistakes; moreover, it could represent an example to easily improve the practical aspects of both pre graduate and postgraduate curricula, and to form more open-minded clinicians, starting both from an ear. Nose and throat (ENT)/audiological and from an Internal Medicine extraction.

Sudden bilateral sensorineural hearing loss as an unusual consequence of accidental ingestion of potassium hydroxide.

OBJECTIVE: To discuss the possible etiopathogenetic mechanism of inner ear damage induced by the ingestion of potassium hydroxide (KOH). CLINICAL PRESENTATION AND INTERVENTION: We report the case of a 37-year-old patient with sudden bilateral sensorineural hearing loss after accidental ingestion of a KOH solution. The first ear, nose and throat examination disclosed only mild edema of the upper airways. He was treated in the intensive care unit and prescribed high-dose steroids, proton pump inhibitors and sucralfate for 2 weeks. Unfortunately, there was no recovery of the hearing loss, and no audiogram changes were noticed after 12 months of follow-up. CONCLUSION: After exploring the possible etiopathogenetic mechanism involved, the authors believe that in this case, a transient severe hemodynamic imbalance can actually be considered to be the most reliable explanation for the inner ear damage and subsequent onset of permanent bilateral sensorineural hearing loss.

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.

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.

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.

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.

Can hypotension episodes cause cochlear damage in young subjects?

In the absence of a satisfactory interpretation, sudden sensorineural hearing loss is often attributed either to infective phenomena or to organic-type circulatory defects. This latter pathogenesis inevitably comes to mind in elderly patients or in the presence of signs of circulatory dysfunction. Nevertheless, the not-infrequent observation of sudden hearing loss in young persons lacking factors predictive of short-term vascular impairment makes us suppose the existence of etiopathogenetic mechanisms of a functional nature. A survey of our cases, matched with a control group, revealed the presence of lower mean blood pressure levels in the group of young patients with 'idiopathic' sudden hearing loss. This finding supports the hypothesis that a condition of haemodynamic imbalance linked to hypotension plays a role in the genesis of cochlear damage in young subjects. Such a mechanism would bear important therapeutic implications: should it be responsible for the damage in at least some cases, vasoactive drugs with a vasodilatative action could have adverse effects on the possibility of recovery.

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.

Is carotid stenosis correlated with tympanosclerosis.

AIM: Tympanosclerosis and atherosclerosis seem to have a similar inflammatory origin despite the different tissues involved. We have investigated the possible relationship between tympanosclerosis and atherosclerosis by evaluating the incidence of tympanic plaques in patients with significant carotid disease. METHODS: Between September 1(st) 2002 and April 30th 2003, we have evaluated all patients undergoing carotid surgery for significant carotid stenosis (group A). If at least 1/5 of the eardrum was occupied by plaques, then the case was considered positive. A control group of patients of similar age and no history of otological nor vascular pathologies was enrolled (group B). The 2 groups were compared statistically by Fisher's test and significance was set at p=0.05. RESULTS: Group A consisted of 84 patients. In this group 32 patients (38.1%) had tympanosclerosis; it was bilateral in 12 cases. Group B had 84 patients. In this group tympanosclerosis was present in 11 cases (13%). The difference between the 2 groups was statistically significant (p=0.005). CONCLUSION: More studies are needed before we can understand the cause of the association between these 2 pathologies. However early screening of atherosclerotic disease in patients with tympanosclerosis seems justifiable on the basis of the data of both the literature and the present study.

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.

Arterial anomalies of the middle ear associated with stapes ankylosis.

An aberrant internal carotid artery in a young woman complaining of pulsatile tinnitus and conductive hearing loss was diagnosed pre-operatively by CT scan and angiographic findings. An exploratory tympanotomy was performed in order to evaluate the cause of the severe conductive hearing loss. It was possible to detect a large persistent stapedial artery associated with a stapedial fixation of unknown cause. Despite these vascular anomalies a stapedotomy was performed successfully.

[Acute epiglottitis in adults: a rare cause of acute respiratory insufficiency]. / L'epiglottite acuta nell'adulto: una rara causa di insufficienza respiratoria acuta.

Acute epiglottitis is an infective disease which may have a rapid and aggressive course to total upper airway obstruction. Due to its rarity (mostly in adults) and aspecific initial clinical features, it may present diagnostic problems to less experienced medical personnel. This is the principal reason why the mortality rate is still unacceptably high. Indirect or direct laryngoscopy is mandatory for diagnosis: as this might be difficult for non-specialist physicians, their awareness of the potentially severe consequences of epiglottic infections is fundamental to minimizing diagnostic delay. In fact, early involvement of experienced ENT and anaesthesiological staff is essential for diagnosis, which must be as prompt as possible and followed by thorough clinical monitoring to avoid fatal outcome.

How a labyrinthine fistula may sometimes be useful for surgery.

The author reports a case of chronic otitis with cholesteatoma complicated by a fistula of the lateral semicircular canal and a stapedo-ovalar ankylosis, probably of otosclerotic origin, in which the decision to take advantage of the fistula to create conditions similar to those generally produced by labyrinthine fenestration allowed virtual functional recovery of the patient's hearing. The case illustrates how, with a ploy of this kind, certain intrinsically negative features may actually turn out to be a blessing in disguise.

Microinjections of alpha 1- and alpha 2-noradrenergic substances in the cerebellar vermis of decerebrate cats affect the gain of the vestibulospinal reflexes.

1. In addition to mossy and climbing fibers, the Purkinje (P)-cells of the cerebellar cortex receive noradrenergic (NA) afferents which originate mainly from the locus coeruleus. Since these fibers impinge also on the vermal cortex of the cerebellar anterior lobe, which receives a labyrinth input and projects to the lateral vestibular nucleus, experiments were performed in precollicular decerebrate cats to find out whether unilateral injection of alpha-adrenergic substances into the vermal cortex of the cerebellar anterior lobe exerted some influence on posture as well as on the dynamic characteristics of vestibulospinal (VS) reflexes evaluated by recording the multiunit EMG responses of the forelimb extensor triceps brachii of both sides to roll tilt of the animal at 0.15 Hz, +/- 10 degrees. 2. Unilateral injection into the vermal cortex of the culmen of the alpha 1-adrenergic agonist metoxamine or the alpha 2-adrenergic agonist clonidine (0.25 microliters at the concentration of 4 micrograms/microliters of saline) produced a postural asymmetry, characterized mainly by a slight decrease of the extensor tonus in the ipsilateral forelimb and an increased tonus in the contralateral forelimb. The same substances significantly increased the gain (imp./sec/deg) of the first harmonic component of the EMG responses of the ipsilateral and the contralateral triceps brachii to animal tilt. The crossed effects were more prominent for the alpha 2- than for the alpha 1-agonist. However, no significant changes in the phase angle of the responses were observed in both instances. The effects described above occurred within 5-10 min after the injection, reached the peak values after 15-20 min, and disappeared within 2 hours. 3. The postural and reflex changes described above were not due to irritative events following the injection, since they were not observed in control experiments after injection of 0.25 microliter of saline into the same corticocerebellar area prior to the administration of the alpha 1- or the alpha 2-adrenergic agonist. Moreover, the resulting effect were dose-dependent. 4. Both the ipsilateral as well as the contralateral effects induced by the alpha 1- or the alpha 2-adrenergic agonist metoxamine or clonidine were impaired by previous injection into the same corticocerebellar area of the corresponding alpha 1- or alpha 2-adrenergic antagonist prazosin or yohimbine, respectively (0.25 microliter at the concentration of 8-16 micrograms/microliters in both cases).(ABSTRACT TRUNCATED AT 400 WORDS)

[The contralateral ear in acquired cholesteatoma in children and adults]. / L'orecchio controlaterale nel colesteatoma acquisito del bambino e dell'adulto.

Contralateral ear pathologies are frequently found in patients suffering from acquired cholesteatoma. A retrospective study was performed on 85 pediatric patients and 105 adults surgically treated for acquired middle ear cholesteatoma. All the patients were checked and the otomicroscopic picture photographed and compared in an attempt to gain insight into the pathogenesis and clinical indications by comparing two samplings from different age groups. In both groups the frequency of pathological contralateral ear manifestations was higher than found in the normal population. Similar results were obtained in the two groups and indicate that the same mechanisms come into play in both ears, starting at infancy. The finding of a particularly common association between cholesteatoma and contralateral retraction pockets with sinus cholesteatoma or pars tensa in the pediatric group appears to confirm that tubal dysfunction plays a pathogenic role in the genesis of cholesteatoma. From the practical point of view, systematic preventative measurement of the contralateral ear can lead to a reduction in major surgery and can affect the choice between open or closed tympanoplasty. Moreover, it may also condition the precision and length of the follow-up.

[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.

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.