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Background and Objectives: Bilateral sequential vestibular neuritis (BSVN) is a rare condition in which an inflammation or an ischemic damage of the vestibular nerve occurs bilaterally in a sequential pattern. We described four cases of BSVN.Subjects and Methods: Every patient underwent video-head impulse test during the first and the second episode of vestibular neuritis (VN), furthermore they have been studied with radiological imaging.Results: Contralateral VN occurred after a variable period from prior event. Vestibular function recovered from the first episode in one case. The other three patients developed contralateral VN. One case was due to a bilateral VN in association with a Ramsay-Hunt syndrome, in another patient clinical records strongly suggested an ischemic etiology, whereas in two cases aetiology remained uncertain. Two patients subsequently developed a benign paroxysmal positional vertigo involving the posterior canal on the side of the latest VN (Lindsay-Hemenway syndrome).Conclusions: Instrumental vestibular assessment represents a pivotal tool to confirm the diagnosis of VN and BSVN.

Purpose: We compared the performance and usability of the Fundus Automated Perimetry (FAP) and Humphrey Field Analyzer (HFA) in patients with glaucoma, ocular hypertension, and healthy subjects. Materials and methods: A total of 60 participants, divided in three groups of 20, glaucoma (POAG), ocular hypertension (OHT), and controls group, underwent a HFA test 24-2 SITA standard and a FAP test 24-2 ZEST sequence, in randomized order. The mean differences between perimeters of mean deviation (MD), pattern standard deviation (PSD) were correlated using the t-test and the Bland–Altman plot while execution time, Glaucoma Staging System 2 (GSS2), Hodapp–Parrish–Anderson staging system, localization of the defect, false positives (FP), and false negatives (FN) were compared with t-test analysis. Usability was measured through answers of a dedicated questionnaire. Results: MD’s difference was higher for FAP than HFA: OHT −2.20 ± 1.33 dB ( p Conclusion: Considering MD and GSS2 classification, FAP finds more severe defects. Moreover, although FAP duration is longer, this method is preferred by most of the patients.

The goal of the present study was to describe a case of perilymphatic fistula (PLF) of the round window (RW) that occurred after sneezing, along with a review of the literature. We report a case of PLF of RW, which was provoked by sneezing, and its consequent medical and surgical treatments. With respect to the review of the literature, articles were initially selected based on their titles or abstracts, followed by methodological evaluation. The patient underwent an explorative tympanotomy (ET) with packaging of RW with the pericondrium, following which the patient's complaints regarding vertigo and imbalance disappeared, but the severe sensorineural hearing loss persisted. For the literature review, five references were selected. These studies showed a great variety in the clinical presentation and healing of symptoms. Sneezing represents a rare but well-recognized cause of PLF, as reported in our case. The correct selection of patients who should undergo ET and an early surgical repair of PLF are mandatory for better outcomes, especially in case of hearing.

Objectives: The development of pneumolabyrinth without previous head trauma is a rare event; the associated symptoms may be nonspecific, and they can simulate various cochleo-vestibular pathological entities. The aim of the present study is to describe one of these rare occurrences, characterized by a peculiar onset. Methods: We report a case of stapes fracture secondary to ear pick penetration into the middle ear with a pneumolabyrinth that caused a recurrent paroxysmal positional vertigo (PPV) mimicking a canalolithiasis. Results: The patient developed a profound left sensorineural hearing loss and an intractable PPV with “migrant” features. A pneumolabyrinth was visualized with high-resolution computed tomography. A perilymphatic fistula (PLF) with stapes fracture was found while performing an explorative tympanotomy. After the surgical treatment of the PLF, the patient no longer complained of vestibular symptoms. Conclusions: To our knowledge, this is the fifth case of traumatic pneumolabyrinth simulating a canalolithiasis without previous history of temporal bone trauma and/or middle ear surgery. A pneumolabyrinth should be suspected in case of patients presenting recurrent intractable PPV after ear trauma.

Canalith jam refers to a condition caused by an otolithic clump blocked inside a semicircular canal, generally provoked by canalith repositioning procedure. We describe the first case of spontaneous canalith jam mimicking an acute vestibular deficit.We report the case of an 82-year-old woman who suffered a sudden episode of persistent rotational vertigo with nausea and vomiting, not provoked by head movements.Videonystagmography revealed a horizontal right-beating spontaneous nystagmus, inhibited by visual fixation. Surprisingly, the positional test showed a direction changing apogeotropic horizontal nystagmus weaker in the left side, compatible with a left side horizontal canal canalolithiasis of the apogetropic type. Returning to the sitting position, a spontaneous nystagmus was observed again, not tilt sensitive. A left side caloric paresis was found.After performing liberatory maneuvers, the spontaneous nystagmus disappeared and a horizontal canal benign paroxysmal positional vertigo of geotropic type was documented. The canal paresis also disappeared.Canalith jam is rarely described and is overall observed as a repositioning manoeuvre complication, not as a mimicker of a vestibular neuritis. Furthermore, our case represents the first observation of a recurrent canalith jam and apogeotropic variant of horizontal canal benign paroxysmal positional vertigo.

A 44-year-old man presented with Acanthamoeba and Fusarium coinfection keratitis. Fifteen years before, he had hyperopic laser in situ keratomileusis, and the coinfection was under the flap. The keratitis was nonresponsive to topical and systemic therapy; therefore, accelerated corneal crosslinking (CXL) with photoactivated riboflavin (photoactivated chromophore for keratitis) was performed to prevent a corneal perforation. After the treatment, there was unexpected rapid corneal melting with subsequent perforation. A reconstructive penetrating keratoplasty was promptly performed and was effective in resolving the coinfection. The current protocol for the application of photoactivated riboflavin CXL should be improved, and clinical criteria for applying this technique are required. Photoactivated riboflavin CXL might be an effective alternative to conventional agents in some cases of fungal and/or amoeba keratitis. However, in cases of deep stromal infections on previous surgically treated anterior stroma, it should not be considered a viable option.

Objective.Anti-TNF-α agents have significantly changed the management of juvenile idiopathic arthritis (JIA). We evaluated the safety and efficacy of adalimumab (ADA) and infliximab (IFX) for the treatment of JIA-associated uveitis in patients treated for ≥ 2 years.Methods.Patients with JIA-associated uveitis treated with IFX and ADA were managed by a standardized protocol and data were entered in the ORCHIDEA registry. At baseline, all patients were refractory to standard immunosuppressive treatment or were corticosteroid-dependent. Data recorded every 3 months were uveitis course, number/type of ocular flares and complications, drug-related adverse events (AE), and treatment switch or withdrawal. Data of patients treated for ≥ 2 years were analyzed by descriptive statistics.Results.Up to December 2014, 154 patients with ≥ 24 months followup were included in the study. Fifty-nine patients were treated with IFX and 95 with ADA. Clinical remission, defined as the absence of flares for > 6 months on treatment, was achieved in 69 patients (44.8%), with a better remission rate for ADA (60.0%) as compared to IFX (20.3%; p < 0.001). A significant reduction of flares was observed in all patients without difference between the 2 treatment modalities. The number of new ocular complications decreased in both groups but was lower for ADA (p = 0.015). No serious AE were recorded; 16.4% of patients experienced 35 minor AE and the incidence rate was lower with ADA than with IFX.Conclusion.At the 2-year followup, ADA showed a better efficacy and safety profile than IFX for the treatment of refractory JIA-associated uveitis.

The Medical Faculties of the University of Padua (Italy) and the University of Vienna (Austria) preserved two series of wax models, made by the Austrian Johann Nepomuk Hoffmayr at the beginning of the 19th century. These models were created in a period of evolution of both medical specialties and organ pathology, which brought morbid organs at the centre of medical investigation. Ceroplastic was considered a useful tool for didactic and research, as it provided a three-dimensional realistically coloured reproduction of organic lesions. The models represent the typical eye diseases of the period, in particular those affecting external parts, which could be investigated without the need for specific instruments devised for the observation of the inner and posterior anatomy of the eye, at that time not yet available. Even if the nosological categories then employed by Hoffmayr were different from those currently used, it has been possible to find a correspondence thanks to the ophthalmological literature of his period. Ceroplastic started to decline at the end of 19th century, substituted by the much less expensive method of preservation of morbid organs in formalin and by new techniques of investigation of the inner body, such as X-ray.

Congenital cholesteatoma within the tympanic membrane is an uncommon entity, with only few cases being documented. The aetiopathogenesis of this lesion is still unknown; however, when cholesteatoma develops in subjects without any history of previous ear inflammation, as in the case we report here, an embryologic origin is deeply suspected. An acquired origin is hypothesized in patients with a previous history of an inflammatory process of the external or middle ear because of the proliferation of the basal cell layer of the tympanic membrane epithelium. We report a rare case of congenital cholesteatoma of the tympanic membrane in an adult patient and review the literature.

Purpose To evaluate the feasibility of near-infrared (NIR) imaging acquisition in a large sample of consecutive pediatric patients with neurofibromatosis type 1 (NF1), to evaluate the diagnostic performance of NF1-related choroidal abnormalities as a diagnostic criterion of the disease, and to compare this criterion with other standard National Institutes of Health (NIH) diagnostic criteria. Methods A total of 140 consecutive pediatric patients (0-16 years old) affected by NF1 (at least two diagnostic criteria), 59 suspected (a single diagnostic criterion), and 42 healthy subjects (no diagnostic criterion) were consecutively included. Each patient underwent genetic, dermatologic, and ophthalmologic examination to evaluate the presence/absence of each NIH diagnostic criterion. The presence of NF1-related choroidal abnormalities was investigated using NIR confocal ophthalmoscopy. Two masked operators assessed Lisch nodules and NF1-related choroidal abnormalities. Results Neurofibromatosis type 1-related choroidal abnormalities were detected in 72 affected (60.5%) and 1 suspected (2.4%) child. No healthy subject had choroidal abnormalities. Feasibility rate of this sign was 82%. Sensitivity, specificity, and positive and negative predictive values of NF1-related choroidal abnormalities were 0.60, 0.97, 0.98, and 0.46, respectively. Compared with standard NIH criteria, the presence of NF1-related choroidal abnormalities was the third parameter for positive predictive value and the fourth for sensitivity, specificity, and negative predictive value. Compared with Lisch nodules, NF1-related choroidal abnormalities were characterized by higher specificity and positive predictive value. The interoperator agreement for Lisch nodules and NF1-related choroidal abnormalities was 0.67 (substantial) and 0.97 (almost perfect), respectively. The use of this sign moved one patient from the suspected to the affected group (0.5%). Conclusions Neurofibromatosis type 1-related choroidal abnormalities represent a new diagnostic sign in NF1 children. The main advantage of this sign seems the theoretical possibility to anticipate NF1 diagnosis, whereas the main obstacle is the cooperation required by very young patients.

Auditory brainstem-evoked responses (ABR) were recorded from the scalp of 30 normoacoustic insulin-dependent diabetic subjects, aged between 15 and 41 yr (29 ± 7 yr). Three different stimulus repetition rates (11, 37, and 87 cps) were used. The results were compared with those obtained from 20 age- and sex-matched, normoacoustic control subjects. In diabetic patients, metabolic control (mean daily plasma glucose, glycosylated hemoglobin) and the presence of retinopathy, nephropathy, and somatic neuropathy were also investigated. The latencies (ms) of ABR waves were significantly impaired in diabetic subjects as compared with normals. Peripheral transmission time (wave I) and central transmission time (waves I–V) were also significantly delayed in diabetic subjects. Moreover, by increasing stimulus repetition rates, a significant increase in waves I–V shift was observed in diabetic patients. ABR impairment was not related to glucose balance, to the duration of diabetes, or to the presence of the diabetic retinopathy, nephropathy, and somatic neuropathy. In conclusion, diabetic neuropathy is characterized not only by somatic and autonomie nerve dysfunctions, but also by the early involvement of the central nervous system (CNS). ABR recording can represent a useful, noninvasive, simple procedure to detect both acoustic nerve and CNS damage.