Chronic inflammatory demyelinating polyneuropathy: evaluation of the vestibular system with cervical and ocular vestibular evoked myogenic potentials.

PURPOSE: To investigate the possibility of vestibular damage in a group of patients suffering from chronic inflammatory demyelinating polyneuropathy (CIDP) using a diagnostic protocol including the caloric test, C-VEMPs and O-VEMPs. METHODS: Twenty patients suffering from CIDP (mean age 58.5 years, range 33-80 years; 4 women and 16 men) were investigated. To assess any eventual audio-vestibular involvement, all patients of the study underwent pure tone audiometry, Fitzgerald-Hallpike caloric vestibular test, C-VEMPs and O-VEMPs. RESULTS: In 11 patients with CIDP values of both O-VEMPs and C-VEMPs were either absent or abnormal. An absent trace at O-VEMPs testing occurred in 36% of these pathological patients, whereas an increase of n10 latency and amplitude was present in the other 64% . CONCLUSIONS: A specific diagnostic protocol including the caloric test, C-VEMPS, O-VEMPS, could be useful when employed for identifying vestibular damage in CIDP patients.

Eustachian tube evaluation in aviators.

The aim of the study is to evaluate the Eustachian tube functionality in a group of aviators to underline a subclinical dysfunction and the related risk of ear fullness or barotitis. Hypobaric chamber allows to simulate the pressure variation of the flight. This prospective study enrolled 42 aviation pilots, members of the Italian Air Force, to whom were evaluated Eustachian tube functionality by Tubomanometry, patients subjective assessments concerning feasibility of Valsalva's and Toynbee's clinical symptoms, tympanometry, and objective Valsalva before and after exposure to hypobaric chamber. The new Eustachian tube score (ETS-7) was also calculated for each pilot before and after exposure to hypobaric chamber. Results of our examination showed that: before chamber exposition, in 92.8 % of pilots, an ETS-7 of eight or more was found bilaterally. In three (7.2 %) cases, a unilateral ETS-7 ≤ 7 was found and two of those had a positive history. After undergoing the hypobaric chamber session, the evaluation of ETS-7 showed only 19 % of pilots with an ETD score ≤7, in particular three bilateral and five unilateral cases. Three of those pilots were clinically positive: two airmen reported persistent fullness, while the other one had a barotitis. Therefore, the combined use of TMM and ETS-7 before and after hypobaric chamber exposure appears to be a reliable method for assessing the functional capacity of the Eustachian tube in aviators, the stressful effect of flight on it, to exclude subjects at increased risk of ear pain, fullness, or barotrauma.

Audiological and vestibular evaluations in vitiligo patients.

The aim of this paper was to investigate audiological abnormalities and potential vestibular injury in a sample of vitiligo subjects. Thirty-five patients with non-segmental vitiligo (NSV) were enrolled in the study. They underwent pure tonal audiometry (PTA), vestibular Fitzgerald-Hallpike caloric test, C-VEM, and O-VEMP testing.  The χ2 test and multiple regression analysis were performed. At PTA, 69% of patients presented with bilateral hearing loss, 8% monaural hearing loss, and 23% normal values. Bilateral caloric stimulations were performed and demonstrated that 14% of patients had a monolateral and 9% had a bilateral pathological response. VEMPs analysis showed that  20% of patients had no O-VEMPs response and 3% had no C-VEMPs response. Comparison between the normal values of healthy subjects and NSV patients showed an alteration of VEMPs in 44%. Multiple regression showed no statistical differences. We propose a specific diagnostic protocol employing PTA, bithermal caloric tests, C-VEMP, and O-VEMP testing to evaluate audio-vestibular damage. Our data were concordant with the anatomic-physiological melanocytic distribution and their possible degeneration linked with NSV.

Eustachian Tube Dysfunction in Children With Adenoid Hypertrophy: The Role of Adenoidectomy for Improving Ear Ventilation.

INTRODUCTION: To our knowledge, few papers have addressed preoperative evaluation of the impact of adenoid hypertrophy (AH) on the pathogenesis of eustachian tube dysfunction (ETD) in children with otitis media with effusion (OME). AIM: The aim of this study was 2-fold: first, to evaluate ETD using tubomanometry and Eustachian Tube Score 7 (ETS-7), in a group of children having AH; second, to assess the clinical impact of adenoidectomy on the ETD of these patients. METHODS: Fifty patients, aged 4 to 15 years, underwent adenoidectomy based on various parameters: size of the adenoids causing canal obstruction (grades 1-4), the presence of OME, and recurrent episodes of rhinosinusitis. The function of the eustachian tube was evaluated using ETS-7 before and after surgical treatment. The patients were followed up for 6 months. RESULTS: Forty children presented ETD. Of these, 36 had a grade 4 AH. The preoperative mean value for ETS-7 was 6.62. The mean postoperative ETS-7 score showed a value of 9.60 with a statistical difference compared to the preoperative value (P = .0015). CONCLUSIONS: Adenoid hypertrophy has a high impact on the frequency of ETD. In the patients observed in the present study, the ETS-7 score appeared to be a valid tool for assessing ETD both preoperatively and postoperatively. Adenoidectomy seemed to be effective in improving ETD as well as middle ear ventilation.

Giant Cell Arteritis (GCA): Pathogenesis, Clinical Aspects and Treatment Approaches.

Giant Cell Arteritis (GCA), or Horton's Arteritis, is a chronic form of vasculitis of the large and medium vessels, especially involving the extracranial branches of the carotid arteries, in particular, the temporal artery, with the involvement of the axillary, femoral and iliac arteries too. Arterial wall inflammation leads to luminal occlusion and tissue ischemia, which is responsible for the clinical manifestations of the disease. A substantial number of patients affected by GCA present head and neck symptoms, including ocular, neurological and otorhinolaryngological manifestations. The aim of this article is to present pathogenesis, clinical aspects and treatment approaches of GCA manifestations.

Malleostapedotomy in stapes revision surgery: Is an endoscopic approach possible?

OBJECTIVES/HYPOTHESIS: The purpose of the present study was to show our preliminary results regarding the endoscopic ear surgery application in malleostapedotomy surgery. STUDY DESIGN: Case series. METHODS: Six patients (four females and two males) who underwent endoscopic ear surgery as revision surgery for otosclerosis were enrolled in this study. For endoscopic malleostapedotomy surgery, rigid angled endoscopes at 0 ° and 30 ° with a length of 14 cm and an outer diameter of 3 and 4 mm were used. A superelastic nitinol stapes prosthesis was used to connect the malleus neck to the oval window. RESULTS: During endoscopic ear surgery, a lateral ossicular chain fixation was present in all cases. The mean operative time in the study group was 81 minutes (range, 73-89 minutes). A statistically significant difference between preoperative and postoperative hearing findings was observed (P = .007). No cases of sensorineural hearing loss or persistent vertigo were observed in the study sample. No cases of prosthesis displacement or prosthesis extrusion occurred after a mean follow-up of 11.5 months. CONCLUSIONS: In 100% of treated cases, the endoscope proved to be adequate for the visualization of the malleus handle and incus, and of their possible abnormalities. Despite the difficulty related to endoscopic single-handed work, none of the surgical steps of endoscopic malleostapedotomy were considered not feasible by endoscopic ear surgery. LEVEL OF EVIDENCE: 4. Laryngoscope, 2611-2614, 2018.

Eustachian tube evaluation in patients with obstructive sleep apnea syndrome.

OBJECTIVES: Investigate the presence of Eustachian Tube Dysfunction (ETD) in Obstructive sleep apnea (OSA) patients. Correlate the ET function and the severity of the sleep apnea syndrome. Correlate nasal resistance and nasal mucociliary clearance with the onset of ETD in these patients. MATERIALS AND METHODS: Forty patients affected by OSA were enrolled in our clinical prospective study. Thirty patients were also investigated as a control group. All of the analyzed subjects underwent ENT examination, anterior rhinomanometry and test for mucociliary transport time. To evaluate Eustachian tube function the Eustachian tube score-7 (ETS-7) were employed. RESULTS: Twenty percent of the patients with a diagnosis of OSA had a diagnosis of ETD (ETS-7 ≤ 7) with a statistical difference with control group (p = .03). No differences regarding ETD in OSA sub-groups were found. CONCLUSION: This study showed that ET blockage or delayed openings were found in most adult patients with OSA when evaluated by a sensible score as the ETS-7. Our data suggest that an evaluation of the Eustachian tube function should be performed in patients suffering from obstructive sleep apnea syndrome.

Endoscopic vs Microscopic Approach in Stapes Surgery: Advantages in the Middle Ear Structures Visualization and Trainee's Point of View.

OBJECTIVE: Comparing the endoscopic and microscopic approaches in stapes surgery, and establishing the surgical approach that allows better results in terms of visualization of the middle ear structures and aids assistant's training. MATERIALS AND METHODS: Twenty-one residents from the first to the fifth year of training in Otolaryngology were enrolled in this prospective study. A questionnaire specifically designed to investigate the understanding of middle ear anatomy, the surgical steps, and a personal opinion about endoscopic stapes surgery was submitted to each resident. RESULTS: Statistical differences between the endoscopic and microscopic approaches were evident about identification of stapes and long process of the incus (p=0.03) and stapes footplate and the oval window (p=0.03). No substantial difference between the two surgical approaches emerged regarding the visualization of the tympanic membrane and the other middle ear structures (p>0.5). A substantial difference in favor of the endoscopic approach emerged regarding the identification of the hole creation in the footplate of the stapes. CONCLUSION: Endoscopic stapes surgery could favor an easier understanding of the surgical technique for assistants in training with little knowledge of the anatomy and surgical steps.

Subtotal resection of vestibular schwannoma: Evaluation with Ki-67 measurement, magnetic resonance imaging, and long-term observation.

Purpose The aim of this study was to compare the postoperative clinical and radiological data of patients with vestibular schwannomas who were initially managed by near total resection (NTR) or subtotal resection (STR). The Ki-67 analysis results were compared with tumor regrowth to determine the presence of a correlation between this proliferative index and postoperative tumor regrowth. Study Design Seventeen adult patients (7 male, 10 female) were retrospectively reviewed. Nine (52.9%) and eight (47.1%) patients underwent NTR and STR, respectively. Postoperative clinical and radiological data associated with vestibular schwannoma growth were compared with the Ki-67 immunohistochemical analysis results. Results Evidence of clinically significant regrowth was observed in four (23.5%) patients. Patients who underwent NTR had a lower rate/incidence of tumor regrowth than did patients who underwent STR. Patients with a higher Ki-67 index had the highest tumor regrowth rates. Conclusions Our study indicates that assessment of the Ki-67 index may be useful for determining the probability of regrowth of vestibular schwannomas when only partial removal is accomplished.

Granulomatosis with polyangiitis and facial palsy: Literature review and insight in the autoimmune pathogenesis.

Granulomatosis with polyangiitis (GPA) is an autoimmune systemic necrotizing small-vessel vasculitis associated with the presence of anti-neutrophil cytoplasmic antibodies (ANCA). Oto-neurological manifestations of ANCA-associated vasculitis according to PR3-ANCA positivity and MPO-ANCA positivity are usually reported. Facial nerve palsy is usually reported during the clinical course of the disease but it might appear as the presenting sign of GPA. Necrotizing vasculitis of the facial nerve 'vasa nervorum' is nowadays the most widely accepted etiopathogenetic theory to explain facial damage in GPA patients. A central role for PR3-ANCA in the pathophysiology of vasculitis in GPA patients with oto-neurological manifestation is reported. GPA requires prompt, effective management of the acute and chronic manifestations. Once the diagnosis of GPA has been established, clinicians should devise an appropriate treatment strategy for each individual patient, based on current clinical evidence, treatment guidelines and recommendations.

Vitiligo: Pathogenesis, clinical variants and treatment approaches.

Vitiligo is a common chronic acquired disease of pigmentation whose etiology is unknown, which usually occurs with asymptomatic whitish patch or macule. Although several hypotheses have been proposed in the literature, the leading theory is still the auto-immune etiology linked to specific genetic mutations. Vitiligo can also be associated with several autoimmune diseases, including autoimmune thyroid diseases, alopecia areata, and halo nevi. Sensorineural hearing loss was reported in several vitiligo patients due to a reduction in the number of melanocytes contained in the membranous labyrinth of the inner ear. Because of its complexity, several therapeutic options are available to treat this systemic disease.

Massive Cerebrospinal Fluid Leak of the Temporal Bone.

Cerebrospinal fluid (CSF) leakage of the temporal bone region is defined as abnormal communications between the subarachnoidal space and the air-containing spaces of the temporal bone. CSF leak remains one of the most frequent complications after VS surgery. Radiotherapy is considered a predisposing factor for development of temporal bone CSF leak because it may impair dural repair mechanisms, thus causing inadequate dural sealing. The authors describe the case of a 47-year-old man with a massive effusion of CSF which extended from the posterior and lateral skull base to the first cervical vertebrae; this complication appeared after a partial enucleation of a vestibular schwannoma (VS) with subsequent radiation treatment and second operation with total VS resection.

Posterior reversible encephalopathy syndrome--Insight into pathogenesis, clinical variants and treatment approaches.

Posterior reversible encephalopathy syndrome is a rare clinicoradiological entity characterized by typical MRI findings located in the occipital and parietal lobes, caused by subcortical vasogenic edema. It was first described as a distinctive syndrome by Hinchey in 1996. Etiopathogenesis is not clear, although it is known that it is an endotheliopathy of the posterior cerebral vasculature leading to failed cerebral autoregulation, posterior edema and encephalopathy. A possible pathological activation of the immune system has been recently hypothesized in its pathogenesis. At clinical onset, the most common manifestations are seizures, headache and visual changes. Besides, tinnitus and acute vertigo have been frequently reported. Symptoms can be reversible but cerebral hemorrhage or ischemia may occur. Diagnosis is based on magnetic resonance imaging, in the presence of acute development of clinical neurologic symptoms and signs and arterial hypertension and/or toxic associated conditions with possible endotheliotoxic effects. Mainstay on the treatment is removal of the underlying cause. Further investigation and developments in endothelial cell function and in neuroimaging of cerebral blood flow are needed and will help to increase our understanding of pathophysiology, possibly suggesting novel therapies.