Isolated horizontal canal hypofunction differentiating a canalith jam from an acute peripheral vestibular loss.

OBJECTIVES: To describe a unique case of acute vertigo presenting with spontaneous horizontal nystagmus (SHN) and a clinical picture consistent with right acute peripheral vestibular loss (APVL) in which an isolated hypofunction of a horizontal semicircular canal (HSC) permitted to detect a spontaneous canalith jam and treat the patient accordingly. METHODS: Case report and literature review. RESULTS: A 74-year old woman presented with acute vertigo, left-beating SHN and a clinical picture consistent with right APVL. Nevertheless, vestibular evoked myogenic potentials were normal with symmetrical amplitudes and the video head impulse test (vHIT) revealed an isolated hypofunction of the right HSC. After repeated head shakings, the supine roll test evoked bilaterally a positioning paroxysmal geotropic horizontal nystagmus suggesting benign paroxysmal positional vertigo involving the non-ampullated arm of the right HSC. vHIT and caloric testing confirmed restitution of HSC function after repositioning maneuvers. CONCLUSIONS: In case of acute vertigo with SHN, a complete functional assessment of vestibular receptors and afferents should always be given in order to avoid misdiagnosis. Canalith jam should be considered in case of spontaneous nystagmus and isolated canal hypofunction.

Use of transrenal DNA for the diagnosis of extrapulmonary tuberculosis in children: a case of tubercular otitis media.

The diagnosis of tuberculosis (TB) is difficult in children, especially for smear-negative pulmonary and extrapulmonary TB, which are common at this age. We report an 11-year-old girl with TB otitis media with negative smear microscopy and Xpert MTB/RIF but positive Mycobacterium tuberculosis-specific transrenal DNA (Tr-MTB-DNA) test results and culture for M. tuberculosis.

Dehiscence of the superior semicircular canal: a review of the literature on its possible pathogenic explanations.

The dehiscence of superior semicircular canal is a well-known affection which is able to explain some cases of hearing loss, tinnitus and/or vertigo unexpectedly presenting in adults without previous otologic affections. Although a diagnostic algorithm has been assessed and a surgical therapy has been indicated, the review of the literature shows that a completely satisfactory explanation for the reason why symptoms of a supposed congenital condition only occur in adulthood is still lacking. A pathogenic hypothesis based on the slow metabolism of the bony labyrinth, which could in time result in a prevalence of bone re-absorption on new bone formation leading to a dehiscence, despite some controversial findings could represent a the most reliable explanation for the question.

Management of growing vestibular schwannomas.

Conservative management of small vestibular schwannomas is frequently proposed as most tumours do not grow. Anyway, tumour growth is reported in 30-40 % of the cases, so that surgery is consequently generally proposed. We primarily observed 161 patients affected by unilateral vestibular schwannomas. All patients were examined by means of gadolinium-enhanced magnetic resonance imaging scans. Tumour growth was recorded in 58 cases (35.8 %) and these subjects set up the group of study. Twenty-two (37.9 %) patients were surgically treated; tumour was always completely removed, all patients had normal facial function after surgery and only one patient suffered from a major complication (cerebellar haematoma). Fourteen patients (24.1 %) were submitted to radiotherapy, while one patient was lost at follow-up and another one died because of other medical reasons. Finally, 20 (34.5 %) subjects continued to be observed for different reasons. The mean follow-up period after identification of growth was 6.1 years. Nine tumours continued to grow, nine tumours stopped growing, one tumour grew and then regressed in size and one tumour decreased. Sixty percent of patients with useful hearing at diagnosis preserved it during the entire observation period. In conclusion, most of VS do not grow; in case of tumour growth, a surgical procedure may be suggested and the outcomes are not negatively influenced by the delay of the procedure. But in some cases, patients can still follow the "wait and scan" policy. In fact, only less than half of the growing tumours continued to grow. Moreover, most of the patients continued to retain a useful hearing.

Tinnitus in elderly patients and prognosis of mild-to-moderate congestive heart failure: a cross-sectional study with a long-term extension of the clinical follow-up.

BACKGROUND: The complex mechanism responsible for tinnitus, a symptom highly prevalent in elderly patients, could involve an impaired control of the microcirculation of the inner ear, particularly in patients with poor blood pressure control and impaired left ventricular (LV) function. METHODS: In order to define the relationship between the presence of tinnitus and the severity and clinical prognosis of mild-to-moderate chronic heart failure (CHF) in a large population of elderly patients (N = 958), a cross-sectional study was conducted with a long-term extension of the clinical follow-up. Blood pressure, echocardiographic parameters, brain natriuretic peptide (BNP), hospitalization, and mortality for CHF were measured. Multivariate logistic regression analysis was used to assess the association between the presence of tinnitus and some of the prognostic determinants of heart failure. RESULTS: The presence of tinnitus was ascertained in 233 patients (24.3%; mean age 74.9 ± 6 years) and was associated with reduced systolic and diastolic blood pressure (123.1 ± 16/67.8 ± 9 vs 125.9 ± 15/69.7 ± 9; P = .027/P = .006), reduced LV ejection fraction (LVEF%; 43.6 ± 15 vs 47.9 ± 14%, P = .001), and increased BNP plasma levels (413.1 ± 480 vs 286.2 ± 357, P = .013) in comparison to patients without symptoms. The distribution of CHF functional class was shifted toward a greater severity of the disease in patients with tinnitus. Combined one-year mortality and hospitalization for CHF (events/year) was 1.43 ± 0.2 in patients with tinnitus and 0.83 ± 0.1 in patients without tinnitus, with an adjusted hazard ratio (HR) of 0.61 (95% confidence interval (CI): 0.37 to 0.93, P <.002). CONCLUSIONS: Our preliminary data indirectly support the hypothesis that tinnitus is associated with a worse CHF control in elderly patients and can have some important clinical implications for the early identification of patients who deserve a more aggressive management of CHF.

Chronic non-allergic rhinitis with neutrophils is associated with higher acid exposure time: A pH-impedance monitoring study.

BACKGROUND & AIM: Chronic rhinitis, a diffuse disease with a prevalence of 40%, can be classified in allergic (AR) and non-allergic rhinitis (NAR). Nasal cytology allows for the identification of different NAR sub-types according to the inflammatory cell infiltrate. NAR etiopathogenesis is not well clarified and, for NARNE (non-allergic rhinitis with neutrophils) subtype, gastroesophageal reflux disease (GERD) has been suggested as one of the etiopathogenetic factors. Aim of this study is to evaluate the role of GERD in patients with NARNE. METHODS: Fifty-one consecutive patients referred to our Ear, Nose and Throat (ENT) unit with nasal symptoms and cytology suggestive for NAR, were enrolled in the study. All the patients performed a gastroenterological evaluation, high resolution esophageal manometry and a 24-h pH-Impedance monitoring. RESULTS: Twenty-five (49%) patients tested positive at nasal cytology for NARNE. A pathologic pH-impedance was identified in seven patients (28%) with NARNE, as opposed to only one (4%) with different NAR subtypes. Statistical analysis showed that higher acid exposure time (AET) and weaker post nasal drainage were more common in NARNE vs. other NAR patients. CONCLUSIONS: NARNE strongly correlates with higher AET and refluxes number; thus, NARNE patients should be tested with pH-impedance monitoring in addition to nasal cytology.

Sensorineural hearing loss and celiac disease: a coincidental finding.

BACKGROUND: Celiac disease (CD) can be associated with a variety of extraintestinal manifestations, including neurological diseases. A new neurological correlation has been found between CD and sensorineural hearing loss (SNHL). OBJECTIVE: To verify the association between SNHL and CD, and to establish whether the neurological hearing impairment in CD is related to nonorgan-specific and antineuronal antibodies, as well as the presence of autoimmune disorders. METHODS: A sample of 59 consecutive biopsy- and serologically proven CD patients were studied. Among CD patients, 11 were newly diagnosed and 48 were on a gluten-free diet. Hearing function was assessed by audiometric analysis in all CD patients as well as in 59 age- and sex-matched controls. Patients were tested for a panel of immune markers including nonorgan-specific autoantibodies and antineuronal antibodies. RESULTS: SNHL was detected in five CD patients (8.5%) and in two controls (3.4%). In one patient, the SNHL was bilateral, whereas the remaining four had a monolateral impairment. The prevalence of SNHL was not significantly different between CD patients and controls. At least one of the antibodies tested for was positive in two of the five CD patients with SNHL and in 12 of the 54 CD patients without SNHL. Antineuronal antibodies to central nervous system antigens were consistently negative in the five CD patients with SNHL. Only one of the five CD patients with SNHL had Hashimoto thyroiditis. CONCLUSIONS: SNHL and CD occur coincidentally. Hearing function should be assessed only in CD patients with clinical signs of hearing deficiency.

The possible role of proton pump inhibitors of the homeostasis of the inner ear.

The possibility of a benefit in some cases of inner ear sufferance by using proton pump inhibitors has been considered after a casual observation. The hypothesis is advanced considering the adverse effect of reflux on the eustachian tube function, the possible influence of the latter on inner ear symptoms and, from a more general point of view, the trigger effect which a gastric dysfunction requiring proton pump inhibitors could exert on the sympathetic system. These considerations, deserving a further study, seem to be based on logical assessment and therefore in our opinion deserve to be kept in mind in trying to define inner ear disorders of uncertain origin.

Possible influence on heart rate on tinnitus.

Assuming the possibility of the inner ear damage due to a hemodynamic imbalance essentially due to an abnormal vasomotor regulatory response, the possibility that heart rate (HR) has a correlation with the onset and/or the enhancement of tinnitus is hypothesized. In fact, recent studies have drawn the influence of other factors than blood pressure, in normotensive subjects, in taking part to the regulation of peripheral resistance, outlining the importance of both cardiac output (CO) - which is a function of heart rate (HR) and stroke volume (SV) and SV itself as a dynamic component to baroreflex control of muscle sympathetic nerve activity (MSNA). From this point of view, it could be possible that a condition of bradycardia can enhance tinnitus regardless of its cause, and conversely that a more elevated HR can be related to a relief of this symptom.

Inner ear dysfunction of uncertain origin: a multidisciplinary approach could give something more.

In order to find out any possible cause of an alteration of the vasomotor reactivity which can be responsible for a more or less severe sufferance of the inner ear, announced by the onset or the enhancement of sensorineural hearing loss, tinnitus, and some kind of dizziness and vertigo, a multidisciplinary approach should be considered. The possibility of an influence of hemodynamic imbalance due to hypotensive changes followed by vasomotor changes affecting the microcirculation of the inner ear has already been widely discussed; moreover, an increase in prevalence of tinnitus (which in many cases can be considered as a symptom of sufferance of the inner ear) has been found in subjects submitted to an "aggressive" antihypertensive therapy as well as in patients with severe heart failure, thus demonstrating a relationship between hemodynamic changes and inner ear dysfunction. For the same reason, the research for this mechanism of imbalance could concern other conditions possibly activating an abnormal response of the autonomic nervous system, which could in turn lead to a circulatory impairment of the labyrinth: among these, affections concerning central nervous system, endocrine system, metabolism, renal apparatus and even gastroenteric diseases with a functional component and any other factor which could interfere with vasomotor regulation should be considered. Thus, the absence of reliable causes for a sufferance of the inner ear should not lead to catalogue it as a disorder of "idiopathic" nature, but should represent a reason for a multidisciplinary investigation on all the possible causes of hemodynamic imbalance and/or autonomic dysregulation.

Bone vibration-induced nystagmus is useful in diagnosing superior semicircular canal dehiscence.

OBJECTIVE: To explore vestibular integrity by bone vibration-induced nystagmus (VIN) during bedside examination. Disease or dysfunction of vestibular end organs would reduce or eliminate their contribution to total eye movement response to VIN. BACKGROUND: It is assumed that vibration of the mastoid (at a frequency of 100 Hz) stimulates all vestibular end organs (semicircular canals and otolith structures). Previous studies have described oculomotor responses to vestibular activation by vibratory stimulus. Stimulation of individual semicircular canals produces eye movement in the plane of the stimulated canal. Vibratory stimulation of otolith structures (utricular macula) produces changes in ocular torsional position. Superior semicircular canal dehiscence (SCD) is responsible for nontypical cochleovestibular symptoms. Diagnosis is difficult without resorting to imaging, high-resolution computed tomography (HRCT) being the only tool providing reliable assessment. To avoid unnecessary referral for expensive imaging, it would be useful to have a simple screening test to select cases for HRCT. METHODS: Video eye movements were recorded in complete darkness in patients diagnosed with SCD. The eye movement responses to long-lasting unilateral vibratory stimulation applied to the mastoid surface were evaluated. RESULTS: VIN evoked a response in all cases, mostly demonstrating excitation of the affected side. The pattern of vertical, torsional and horizontal eye velocity and eye position was evaluated with three-dimensional infrared video-oculography (50 Hz sampling). CONCLUSION: Analysis of VIN, recently proposed to study transmission of excitatory stimuli by bone conduction, may be appropriate for altered immittance caused by dehiscence. This promises to be an interesting new field of research.

Otoscopic examination: a diagnostic contribution for atherosclerotic disease?

Tympanosclerosis is a particular kind of chronic inflammatory response of the middle ear to mechanical injuries and/or infections. This condition is characterised by the formation of plaques presenting ultrastructural analogies with the atherosclerotic lesions, extended to the tympanic membrane and possibly to the ossicular chain. The less severe degree of tympanosclerosis is represented by asymptomatic and clinically unsignificant whitish plaques within the tympanic membrane, detectable at otoscopy. The pathogenesis of this phenomenon is supposed to present a tight relationship with the pathogenesis of atherosclerosis. This observation has been already reported in medical literature, but deserves further clinical confirmations to better define the real extent of the analogies of both affections. A practical implication of this matter of study could be the possibility to find out a fast and non-invasive test as an early marker of an increased risk of atherosclerotic disease: could otoscopy play such a role?

Could echocardiography yield a cardiovascular profile of the tinnitus prone subject?

The possible genesis of some damage of the inner ear from a hemodynamic imbalance of functional origin, possibly linked to hypotension followed by an abnormal vasomotor regulatory activity, has been pointed out by our group over the years. As tinnitus, which is often referable to an inner ear origin, can represent a signal of incoming sufferance of the organ of Corti and may not necessarily be linked to hearing impairment, it seemed of some utility to investigate on the prevalence of tinnitus under various well monitored hemodynamic conditions. This led to observe that the prevalence of this symptom, regardless of audiological features, was increased under "aggressive" antihypertensive therapy as well as in particularly severe degree of heart decompensation. These data represent a first step and encourage in searching for a profile of subject who could be more prone to the development of tinnitus with respect to the normal population, even in absence of pathological conditions. With this aim, echocardiography is thought to be able to yield useful informations in addition to standard and ambulatory blood pressure monitoring, in order to obtain a better definition of the correlations between cardiovascular function (and related changes) and inner ear insufficient perfusion.

Superior canal dehiscence with tegmen defect revealed by otoscopy: Video clip demonstration of pulsatile tympanic membrane.

Superior canal dehiscence is a pathologic condition of the otic capsule acting as aberrant window of the inner ear. It results in reduction of inner ear impedance and in abnormal exposure of the labyrinthine neuroepithelium to the action of the surrounding structures. The sum of these phenomena leads to the onset of typical cochleo-vestibular symptoms and signs. Among them, pulsatile tinnitus has been attributed to a direct transmission of intracranial vascular activities to labyrinthine fluids. We present the first video-otoscopic documentation of spontaneous pulse-synchronous movements of the tympanic membrane in two patients with superior canal dehiscence. Pulsating eardrum may represent an additional sign of third-mobile window lesion.

Technique and Preliminary Analysis of Drug-Induced Sleep Endoscopy With Online Polygraphic Cardiorespiratory Monitoring in Patients With Obstructive Sleep Apnea Syndrome.

Importance: Drug-induced sleep endoscopy is a diagnostic technique that allows dynamic evaluation of the upper airway during artificial sleep. The lack of a standardized procedure and the difficulties associated with direct visual detection of obstructive events result in poor intraobserver and interobserver reliability, especially when otolaryngology surgeons not experienced in the technique are involved. Objectives: To describe a drug-induced sleep endoscopy technique implemented with simultaneous polygraphic monitoring of cardiorespiratory parameters (DISE-PG) in patients with a diagnosis of obstructive sleep apnea syndrome and discuss the technique's possible advantages compared with the standard procedure. Design, Setting, and Participants: This prospective cohort study included 50 consecutive patients with obstructive sleep apnea syndrome who underwent DISE-PG from March 1, 2013, to June 30, 2014. A standard protocol was adopted, and all the procedures were carried out in an operation room by an experienced otolaryngology surgeon under the supervision of an anesthesiologist. Endoscopic and polygraphic obstructive respiratory events were analyzed offline in a double-blind setting and randomized order. Main Outcomes and Measures: The feasibility and safety of the DISE-PG technique, as well as its sensitivity in detecting respiratory events compared with that of the standard drug-induced sleep endoscopy procedure. Results: All 50 patients (43 men and 7 women; mean [SD] age, 51.1 [12.1] years) underwent DISE-PG without technical problems or patient difficulties regarding the procedure. As expected, polygraphic scoring was more sensitive than endoscopic scoring in identifying obstructive events (mean [SD] total events, 13.3 [6.8] vs 5.3 [3.6]; mean [SD] difference, 8.8 [5.6]; 95% CI, 7.3 to 10.4; Cohen d, -1.5). This difference was most pronounced in patients with a higher apnea-hypopnea index (AHI) at baseline (mean [SD] difference for AHI >30, 27.1% [31.0%]; 95% CI, -36.2% to 90.4%; Cohen d, 0.2; for AH I >40, 76.0% [35.5%]; 95% CI, 4.6% to 147.4%; Cohen d, 0.5; for AHI >50, 92.2% [37.2%]; 95% CI, 17.3% to 167.1%; Cohen d, 0.6) and a high percentage of hypopneas (≥75% of all obstructive events) at baseline (mean [SD] difference, 20.2% [5.4%]; 95% CI, 9.2% to 31.3%; Cohen d, 1.1). No other anthropomorphic or polygraphic features at baseline were associated with the differences between the DISE-PG and baseline home sleep apnea test. Conclusions and Relevance: The DISE-PG technique is feasible, safe, and more sensitive at detecting an obstructed breathing pattern than is drug-induced sleep endoscopy alone. The DISE-PG technique could be helpful for accurate comprehension of upper airway obstructive dynamics (ie, degree of obstruction and multilevel pattern) and a nonobstructive breathing pattern (ie, central apneas).

Is tinnitus useful in early detection of incoming heart decompensation?

In previous studies the possibility of a mechanism of hemodynamic imbalance consequent to a sharp decrease in blood pressure causing a more or less transient inner ear impairment was evaluated and to some extent demonstrated. In this scenario, tinnitus is very frequently present as a debilitating symptom associated to cochlear impairment. On the other hand, a possible cause of a sharp decrease in blood pressure is represented by major cardiovascular disorders, requiring an early assessment in order to avoid fatal consequences. In this paper, the hypothesis of resorting to tinnitus as a simple warning method for self detecting a possibly incoming cardiovascular imbalance in patients with heart failure (HF) is advanced.

Is there a role for atrial peptides in the labyrinthine "disease"?

The important role of atrial natriuretic peptides (ANP) in regulating blood pressure and changing vascular permeability has been widely studied and assessed during the last ten years. Considering the characteristics of this mechanism, which is responsible for a hypotensive and hypovolemic effect, and the possible role of hypotension associated with a default of autoregulatory sympathetic reactions in inner ear unexplained disorders, it seems reasonable to hypothesize a possible involvement of ANP system in the genesis of such disorders. As a matter of fact, the presence of specific receptors for ANP in the inner ear has been widely reported in studies concerning both rat and human inner ear, although their precise role in the labyrinthine homeostasis has not been satisfactory explained until now. Some aspects concerning vascular and fluid regulation of the inner ear under different conditions still remain not totally clear, and consequently a detailed explanation to the possible mechanism causing inner ear disorders of functional origin is lacking; from this point of view, an investigation on the serum level of ANP in subjects with labyrinthine affections of uncertain origin could be of some utility in contributing to assess the role of this system in the inner ear fluid regulation and in the inner ear perfusion and to investigate on the possible influence of an abnormal ANP release in some kind of inner ear damage.