Giancarlo Altissimi

Churg–Strauss syndrome

Churg-Strauss syndrome (CSS), alternatively known as eosinophilic granulomatosis with polyangiitis (EGPA), was first described in 1951 by Churg and Strauss as a rare disease characterized by disseminated necrotizing vasculitis with extravascular granulomas occurring exclusively among patients with asthma and tissue eosinophilia. EGPA is classified as a small-vessel vasculitis associated with antineutrophil cytoplasmic antibodies (ANCAs) and the hypereosinophilic syndromes (HESs) in which vessel inflammation and eosinophilic proliferation are thought to contribute to organ damage. Although still considered an idiopathic condition, EGPA is classically considered a Th2-mediated disease. Emerging clinical observations provide compelling evidence that ANCAs are primarily and directly involved in the pathogenesis of AASVs, although recent evidence implicates B cells and the humoral response as further contributors to EGPA pathogenesis. EGPA has traditionally been described as evolving through a prodromic phase characterized by asthma and rhino-sinusitis, an eosinophilic phase marked by peripheral eosinophilia and organ involvement, and a vasculitic phase with clinical manifestations due to small-vessel vasculitis. The American College of Rheumatology defined the classification criteria to distinguish the different types of vasculitides and identified six criteria for EGPA. When four or more of these criteria are met, vasculitis can be classified as EGPA. The French Vasculitis Study Group has identified five prognostic factors that make up the so-called five-factor score (FFS). Patients without poor prognosis factors (FFS=0) have better survival rates than patients with poor prognosis factors (FFS≥1). The treatment of patients with CSS must be tailored to individual patients according to the presence of poor prognostic factors. A combination of high-dose corticosteroids and cyclophosphamide is still the gold standard for the treatment of severe cases, but the use of biological agents such as rituximab or mepolizumab seems to be a promising therapeutic alternative.

Distortion-product otoacoustic emissions and glycerol testing in endolymphatic hydrops

Objectives/Hypothesis Aural fullness is a frequent symptom of endolymphatic hydrops. Its evaluation may provide valuable information on the initial stage of development of endolymphatic hydrops.

Distortion product otoacoustic emissions in Ménière's disease

Distortion product otoacoustic emissions (DPOAEs) are an objective, non-invasive measure for evaluating outer hair cell (OHC) activity. In this study DPOAEs were measured in 70 patients affected by Ménières disease (MD). In addition 58 out of 70 patients performed both an audiometric threshold evaluation pre- and post-glycerol administration (i.e. glycerol test) and DPOAEs pre- and post-osmotic drug assumption. The purpose of this combined form of testing was to explore the effects of glycerol on the active non-linear mechanisms of the cochlea. More than 60% of the ears with MD emitted DPOAEs despite the presence of an average hearing threshold level above 40 dB (HTL). Changes in the DPOAE baseline measures were observed in 32.4% of cases after glycerol administration. Patients were divided in four groups according to the different pattern of DPOAEs shown after the glycerol test. In particular, three-quarters of cases showed a significant increase in DPOAE amplitude, one-quarter of patients, who initially did not express DPOAEs, eventually did after intake of the osmotic agent, while no decreased DPOAEs were observed in these series. The different expressions of DPOAEs should be associated with the evolutive phases of MD. In addiction, elucidation of the relationship between DPOAEs and the stages of MD was an outcome of this test.

The Tinnitus Handicap Inventory as a Screening Test for Psychiatric Comorbidity in Patients with Tinnitus

BACKGROUND Psychiatric comorbidity is common in patients who seek help for tinnitus. The perceived severity of tinnitus correlates closer to psychological and general health factors than to audiometrical parameters. Audiologists need valid screening tools in order to identify patients with psychiatric disorders and to tailor treatment in a multidisciplinary setting. The tinnitus handicap inventory (THI) has gained widespread acceptance as a self-report measure of tinnitus handicap. In several studies, THI test score correlates with the level of psychopathologic distress. The aim of our study was to investigate the predictive power of the test THI in relation to psychiatric disorders. METHODS We recruited 156 patients with chronic tinnitus who have requested help at our tinnitus center. All patients underwent psychiatric evaluation, the diagnosis was made in agreement with the DSMIV-TR criteria; all patients filled out the following questionnaires: THI, Symptomatic Check List-90-Revised (SCL-90-R) and Stress-related Vulnerability Scale (VRS). Sensibility and specificity of tests as screening tool for psychiatric disorders was evaluated using Receiver Operating Characteristic (ROC) curve. RESULTS Sixty-eight patients (prevalence: 43.59%) were found to be affected by a psychiatric disorder. AUC = 0.792 (p > 0.001) shows that THI is a moderately accurate test to individuate psychiatric affected people among our sample. We identified a score of 36 at THI (sensibility = 86.76%; specificity = 59.09%) as an appropriate cut-off point. CONCLUSIONS If a patient reports a THI score greater than 36, the audiologist should supplement diagnostic studies with a psychiatric evaluation.

Tinnitus: clinical experience of the psychosomatic connection

Background The connection between psychopathology and tinnitus is complex and not adequately studied. The aim of this study is to investigate the relationship between tinnitus and psychiatric comorbidities from different points of view: categorical, dimensional, temperamental, and perceived stress level. Methods Two hundred and thirty-nine patients affected by tinnitus were recruited between January and October 2012. Patients underwent a preliminary battery of tests including the Tinnitus Handicap Inventory (THI), Symptom Check List (SCL90-R), Temperament and Character Inventory (TCI), and Stress-Related Vulnerability Scale (VRS), and eventually a full psychiatric evaluation. Results One hundred and fourteen patients (48% of the total sample) presented psychiatric comorbidity. Among these, a higher prevalence of depression, somatization, obsession, and anxiety was found. More than 41% of patients affected by decompensated tinnitus reported a family history of psychiatric disorders. Significant positive correlations between the psychopathological screening tools (SCL90-R and VRS) and THI were found. Patients affected by comorbid psychiatric disorder showed specific temperamental and characterial predispositions. Conclusion Psychiatric comorbidity in subjects affected by tinnitus is frequent. Stress can be considered as a factor leading to damage and dysfunction of the auditory apparatus. The vulnerability to neurotic disorders and the lack of coping capabilities can play a critical role in the clinical history of patients affected by severe tinnitus.

Tinnitus Holistic Simplified Classification (THoSC): A New Assessment for Subjective Tinnitus, With Diagnostic and Therapeutic Implications.

Objective: One of the most debated topics in tinnitus is its standard and practical classification. The most popular classification distinguishes subjective from objective tinnitus. Other classifications are based on different features. On the whole, they seem incomplete, and the diagnostic and therapeutic algorithms are often difficult for practical purposes. The aim of this work is to develop a new diagnostic and therapeutic algorithm. Methods: Our model is based on 10 years of experience. In particular, the starting point is the data retrieved from 212 consecutive patients in our Tinnitus Unit between May and December 2013: We found a clear auditory disorder in 74.5% of the population, muscolo-skeletal disorders and/or trigeminal disease in 57.1%, and psychiatric comorbidities in 43.8%. Different features coexisted in 59.9% of the population. Results: Following such data we propose the Tinnitus Holistic Simplified Classification, which takes into account the different tinnitogenic mechanisms and the interactions between them. It differentiates tinnitus that arises from: (1) auditory alterations (Auditory Tinnitus), (2) complex auditory-somatosensory interactions (Somatosensory Tinnitus), (3) psychopathological-auditory interactions (Psychopathology-related Tinnitus), and (4) 2 or all of the previous mechanisms (Combined Tinnitus). Conclusions: In our opinion this classification provides an accurate and easy tailored path to manage tinnitus patients.

Somatosensory Tinnitus: Correlation between Cranio-Cervico-Mandibular Disorder History and Somatic Modulation

In a subpopulation of patients, tinnitus can be modulated by movements of the jaw or head and neck due to complex somatosensory-auditory interactions. In some of these subjects, tinnitus could be related to an underlying temporomandibular (TMJ) or craniocervical (NECK) dysfunction that, if correctly identified, could streamline treatment and increase chances of tinnitus improvement. However, it is still unclear whether somatic modulation of tinnitus could be used as a screening tool for identifying such patients. In this study, we included 310 tinnitus patients with normal hearing, no psychiatric comorbidities, and a positive history of TMJ and/or NECK dysfunction and/or a positive modulation of tinnitus to evaluate the characteristics of somatic modulation, investigate the relationship between positive history and positive modulation, and identify factors most strongly associated with somatic modulation. Tinnitus modulation was present in 79.67% of the patients. We found a significant association within the same subjects between a positive history and a positive tinnitus modulation for the same region, mainly for TMJ in unilateral tinnitus patients and for TMJ + NECK in bilateral tinnitus patients. A strong correlation between history and modulation in the same somatic region within the same subgroups of subjects was also identified. Most TMJ maneuvers resulted in an increased loudness, while NECK maneuvers showed an increase in tinnitus loudness in about 59% of cases. High-pitched tinnitus and male gender were associated with a higher prevalence of modulation; no differences were found for tinnitus onset, Tinnitus Handicap Inventory score, and age. In this paper, we report a strong association between history and modulation for the same regions within the same patients; such an association should always be investigated to improve chances of a correct diagnosis of somatosensory tinnitus.

Somatosensory tinnitus: Current evidence and future perspectives:

In some individuals, tinnitus can be modulated by specific maneuvers of the temporomandibular joint, head and neck, eyes, and limbs. Neuroplasticity seems to play a central role in this capacity for modulation, suggesting that abnormal interactions between the sensory modalities, sensorimotor systems, and neurocognitive and neuroemotional networks may contribute to the development of somatosensory tinnitus. Current evidence supports a link between somatic disorders and higher modulation of tinnitus, especially in patients with a normal hearing threshold. Patients with tinnitus who have somatic disorders seems to have a higher chance of modulating their tinnitus with somatic maneuvers; consistent improvements in tinnitus symptoms have been observed in patients with temporomandibular joint disease following targeted therapy for temporomandibular disorders. Somatosensory tinnitus is often overlooked by otolaryngologists and not fully investigated during the diagnostic process. Somatic disorders, when identified and treated, can be a valid therapeutic target for tinnitus; however, somatic screening of subjects for somatosensory tinnitus is imperative for correct selection of patients who would benefit from a multidisciplinary somatic approach.

Relationship between hearing function and myasthenia gravis: A contemporary review:

There is increasing evidence of a connection between hearing function and myasthenia gravis (MG). Studies of the pathophysiological basis of this relationship suggest that acetylcholine receptors (AChRs) on outer hair cells (OHCs) play a central role. In patients with MG, autoantibodies against AChRs induce a progressive loss of AChRs on OHCs, decreasing their electromotility. The stapedial reflex decay test can be altered in MG patients, and can be used as an additional tool for diagnosis and monitoring. Transient evoked and distortion product otoacoustic emissions are the main diagnostic tool for monitoring OHC functionality in MG patients, and can be used to record subclinical hearing alterations before the onset of clinically evident hearing loss. Understanding the association between MG and hearing dysfunction requires a multidisciplinary approach. Otolaryngologists should take this relationship into account when approaching patients with a diagnosis of myasthenia gravis and “in patients with MG” with ण128;œin MG patients, and the progress of hearing alterations should always be monitored in patients with MG.

Suppression tuning characteristics of 2f1-f2 distortion product otoacoustic emissions

The 2f1-f2 distortion product otoacoustic emission (DPOAE) is suppressed by a third tone following a frequency selective pattern and outlining tuning curves which are generally similar to neural and psychophysical findings. The most effective suppressor tone lies between f1 and f2; less suppressive effects are produced by an added tone that lies in the 2f1-f2 site. The slope of iso-suppression curves is much steeper on the higher flank than on the lower flank of the suppression curve (respectively 100-115 dB/octave and 25-35 dB/octave). A suppression fine structure can be observed in the region of the tip especially for higher DPs probably connected with the fine structure of distortion products and with the instability of cochlear activity. A saturation point, around 70 dB or more has been evidenced on the growth rate functions. Continuous and interrupted suppressor presentation can induce some differences in the responses. Suppression of DPOAEs can provide a wider knowledge on active non-linear mechanisms in the cochlea, and on frequency selectivity also in a clinical context.