Giannicola Iannella

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.

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.

Vestibular neurolabyrinthitis: a follow-up study with cervical and ocular vestibular evoked myogenic potentials and the video head impulse test.

Objectives: The aim of this study was to evaluate prospectively, in a group of patients affected by vestibular neurolabyrinthitis (VN), a diagnostic protocol including cervical vestibular evoked myogenic potentials (C-VEMPs), ocular vestibular evoked myogenic potentials (O-VEMPs), and the video head impulse test (vHIT). Methods: The diagnosis of VN was based on the patient’s clinical history, an absence of associated auditory or neurologic symptoms, and a neuro-otological examination with an evaluation of lateral semicircular canal function by use of the Fitzgerald-Hallpike caloric vestibular test and the ice test. Results: In our series, 55% of the cases were superior and inferior VN, 40% were superior VN, and 5% were inferior VN. These cases, however, comprised different degrees of vestibular involvement, as the individual vestibular end organs have different prognoses. Four patients had only deficits of the horizontal and superior semicircular canals or their ampullary nerves. Conclusions: The implementation of C-VEMPs, O-VEMPs, and the vHIT in a vestibular diagnostic protocol has made it possible to observe patients with ampullary VN in a way that has not been feasible with other types of vestibular examinations. The age of the patient seems to have some impact on recovery from VN. When recovery occurs in the utricular and saccular nerves first and in the ampullary nerves subsequently, it may be reasonable to expect a more favorable outcome.

Selective Vestibular Neurolabyrinthitis of the Lateral and Superior Semicircular Canal Ampulla and Ampullary Nerves

Objectives: The diagnosis of vestibular neurolabyrinthitis is based on the sudden appearance of vertigo that lasts for hours or days without associated cochlear or central nervous system signs or symptoms. The advent of the video head impulse test, cervical vestibular evoked myogenic potential testing, and ocular vestibular evoked myogenic potential testing has provided interesting clinical evidence for evaluating and monitoring the damage to specific compartments of the vestibular apparatus. These various methods of testing individual end-organ functions may have a clinical impact on the vestibular workup of neurolabyrinthitis. Methods: This report describes 3 patients with acute vestibular neurolabyrinthitis in whom caloric tests, cervical vestibular evoked myogenic potentials, ocular vestibular evoked myogenic potentials, and the video head impulse test led to a suspicion of peripheral vestibular deficits of the lateral or superior semicircular canal ampulla or ampullary nerves. Results: In our patients, the examination results (normal hearing, absence of responses on caloric testing, and bilateral preservation of cervical and ocular vestibular evoked myogenic potentials) disclosed an acute partial superior vestibular neurolabyrinthitis. Conclusions: To our knowledge, these are the first reported cases in which selective damage to the lateral and superior semicircular canals and their nerves caused by neurolabyrinthitis was demonstrated clinically. Our clinical results indicate that the damage can be selective for specific vestibular end organs.

Endoscopic Versus Microscopic Approach in Stapes Surgery: Are Operative Times and Learning Curve Important for Making the Choice?

Objective: Analyze the surgical outcomes of endoscopic stapes surgery, comparing the results with a conventional stapes surgery under microscopic approach. Estimate the operation type of each surgical approach and show a learning curve of endoscopic stapes surgery. Study Design: Retrospective study. Setting: Tertiary referral center. Patients: Twenty patients who underwent endoscopic stapedotomy for otosclerosis and 20 patients who underwent microscopic stapedotomy for otosclerosis. Interventions: Endoscopic and microscopic stapes surgery. Main Outcome Measures: Operating time, preoperative and postoperative hearing, intraoperative findings, postoperative complications, and postoperative pain. Results: The group of patients who underwent endoscopic stapes surgery showed a mean operative time calculated to be 45.0 min. The group of patients treated by microscopic approach had an estimated mean value of 36.5 min. Statistical difference was evident (p value = 0.01). The average duration of endoscopic surgery varied as the surgeon gained experience. There were no statistical differences between the average surgical times for the endoscopic and microscopic approaches (p >0.05) in the last 4-month period of surgery. Through the endoscopic approach the percentage of ears with a postoperative air-bone gap ⩽20 dB was 95%. No difference from the percentage of the microscopic group (90%) (p >0.05) was reported. No difference regarding the incidence of intraoperative findings and postoperative complications between endoscopic and microscopic approaches was found. Conclusion: Audiological outcomes achieved by endoscopic surgery are similar to the results obtained through a microscopic approach. Longer initial operative times and a learning curve are the principal grounds that might discourage most ear-surgeons from commencing endoscopic stapes surgery.

Giant Petrous Bone Cholesteatoma: Combined Microscopic Surgery and an Adjuvant Endoscopic Approach.

Petrous bone cholesteatomas (PBCs) are epidermoid cysts, which have developed in the petrous portion of the temporal bone and may be congenital or acquired. Cholesteatomas arising in this region have a tendency to invade bone and functional structures and the middle and posterior fossae reaching an extensive size. Traditionally, surgery of a giant PBC contemplates lateral transtemporal or middle fossa microscopic surgery; however, in recent years, endoscopic surgical techniques (primary or complementary endoscopic approach) are starting to receive a greater consensus for middle ear and mastoid surgeries. We report the rare case of an 83-year-old Caucasian male affected by a giant cholesteatoma that eroded the labyrinth and the posterior fossa dura and extended to the infralabyrinthine region, going beyond the theca and reaching the first cervical vertebra. The giant cholesteatoma was managed through a combined approach (microscopic and, subsequently, complementary endoscopic approach). In this case report, we illustrate some advantages of this surgical choice.

Taste and Olfaction in Middle Ear Surgery

Objective: The aim of this study was to assess pre- and postoperative taste ability in patients undergoing middle ear surgery for otosclerosis or chronic otitis media. Olfactory function was also evaluated to rule out taste deficits due to concomitant nasal pathology. Methods: All patients underwent ear, nose, and throat examination, otomicroscopy, nasal endoscopy, anterior rhinomanometry, taste testing, and olfactory testing. Patients were evaluated at 1 to 5 days preoperatively (T0), and at 1 (T1), 6 (T6), and 12 (T12) months postoperatively. Results: Both groups of patients experienced worsening of the mean taste threshold postoperatively. This phenomenon was more serious in poststapedotomy patients. Follow-up showed progressive improvement in both groups. All values of olfactory testing were within the normal range for otosclerosis patients. Patients with chronic otitis media showed variable postoperative findings. Conclusion: Chorda tympani function can be negatively affected by middle ear surgery. Deficits may be more marked in stapedotomy patients than in those undergoing tympanoplasty. Postoperative recovery of taste is satisfactory, although with different timelines for the 2 types of pathology.

Retinitis pigmentosa: evaluation of the vestibular system with cervical and ocular vestibular evoked myogenic potentials and the video head impulse test.

Objective Retinitis pigmentosa (RP) represents a group of inherited disorders in which abnormalities of the photoreceptors lead to progressive visual loss. Night blindness, peripheral visual field loss, and eventual total blindness represent typical visual damage of such disease. No study has previously evaluated the presence of a “latent” vestibular deficit in patients with RP. Study Design Prospective study with caloric test, cervical vestibular evoked myogenic potentials (C-VEMPs), ocular vestibular evoked myogenic potentials (O-VEMPs), and video head impulse test (v-HIT). Setting Tertiary referral center. Patients 16 patients suffering from RP. Intervention Evaluation of vestibular dysfunction with caloric test, C-VEMPs, O-VEMPs, and the measurement of the vestibular-ocular reflex (VOR) using the v-HIT. Results Only five patients with RP showed normal values in all the vestibular tests performed. Three patients had an evident deficit at the caloric test, whereas eight (50%) of them had a normal caloric test but a pathological response in at least one of the other vestibular tests performed. No patient of the study showed a bilateral otolith or ampullary dysfunction. Conclusion Our patients with RP unexpectedly showed pathological responses in at least one of the vestibular tests performed. Nowadays, in patients affected by RP, a vestibular diagnostic protocol must include VEMPs and v-HIT to confirm the vestibular damage and to identify selective damage of the vestibular nerve.

Investigation of pepsin in tears of children with laryngopharyngeal reflux disease

OBJECTIVES Numerous investigations postulated that laryngopharyngeal reflux (LPR) is implicated in the pathogenesis of various upper airway inflammatory diseases as sinusitis or dacryostenosis. The presence of pepsin in tears might be confirmed the presuntive hypothesis of the arrival in the nasolacrimal ducts and precorneal tears film through the laryngopharyngeal reflux of either gastric acid or stomach secretions (pepsin) with inflammatory potentialities. The aim of this preliminary study was to identify the presence or absence of pepsin in the tears collected from children with a high suspicion of LPR who underwent 24-h pH (MII-pH) monitoring to confirm the disease. METHODS This study enrolled 20 patients suffering from symptoms of laryngopharyngeal reflux that underwent 24-h multichannel intraluminal impedance (MII)-pH monitoring to confirm the disease. The findings of the study group were compared with those of a control group of patients with negative pH monitoring. The quantitative analysis of human pepsin concentration in the tear samples was performed by ELISA method in both groups. RESULTS Four children (20%) of the study group showed pepsin in the tears. All of the subjects belonging to the control group were negative for its presence. No difference differences in the total number of reflux episodes and the number of weakly basic reflux in the pepsin positive patients vs. pepsin negative children were present. CONCLUSIONS 20% of the children with diagnosed LPR showed pepsin in the tears. Our specific investigation might provide information regarding sinusitis or dacryostenosis.

Endoscopic versus microscopic approach in attic cholesteatoma surgery

PURPOSE Compare the outcomes of primary exclusive endoscopic ear surgery with those of the microscopic ear surgery in a group of patients affected by attic cholesteatoma. MATERIALS AND METHODS Eighty patients suffered from attic cholesteatoma. Forty patients surgical treated with endoscopic ear surgery and forty patients surgical treated with microscopic ear surgery. RESULTS No statistical differences were found in the parameters analysed (frequency of facial nerve dehiscence, age, disease duration, site of dehiscence) between the endoscopic and microscopic groups indicating a homogeneous selection thus providing a good comparison of the outcomes between the two groups. None of the patients in our survey developed postoperative iatrogenic facial palsy. Graft success rate was 100% in both groups. The overall operation time of endoscopic ear surgery presented a mean value of 87.8min, while in the group of patients treated via microscopic ear surgery a lower mean value of 69.9min was reported. The average healing time was 36.3days for the endoscopic subgroup and 47.8days for the microscopic subgroup. CONCLUSION The surgical outcomes of endoscopic ear surgery are comparable to those of the conventional approach in terms postoperative air-conduction, graft success rate and taste sensation. The analysis of postoperative pain and healing times showed better results for EES. The mean operative times of endoscopic ear surgery gradually shortened as the surgeons gained expertise in performing one-hand surgery.