Posterior Semicircular Canal Plugging Relieves Tumarkin's Crisis in Ménière's Disease Patients.

(1) Background: Patients affected by Ménière's disease can experience Tumarkin's syndrome, which is characterized by postural instability, gait abnormalities, and, occasionally, an abrupt loss of balance known as vestibular drop attack or Tumarkin's crisis. In this study, semicircular canal plugging is proposed as the definitive treatment for this condition. The outcomes of this type of surgery are discussed. (2) Methods: A total of 9 patients with a confirmed diagnosis of Ménière disease suffering from Tumarkin crisis underwent posterior semicircular canal plugging. These patients were assessed with Video Head Impulse Tests, vestibular evoked myogenic potentials, and Pure Tone Audiometry preoperatively and postoperatively. (3) Results: VHIT showed a postoperative decrease in PSC gain median (Preop. 0.86 and postop. 0.52; p < 0.009). No statistically significant differences were described for the anterior semicircular canal and the lateral semicircular canal. No patient experienced new Tumarkin crisis after the surgical treatment. (4) Conclusions: Our ten years of experience with posterior semicircular canal plugging in Ménière disease patients with Tumarkin's syndrome has shown that this type of surgical procedure is successful in controlling Tumarkin's crisis, with high patient satisfaction and little worsening in hearing level.

Posterior Semicircular Canal Dehiscence with Vestibulo-Ocular Reflex Reduction for the Affected Canal at the Video-Head Impulse Test: Considerations to Pathomechanisms.

Posterior semicircular canal dehiscence (PSCD) has been demonstrated to result in a third mobile window mechanism (TMWM) in the inner ear similar to superior semicircular canal dehiscence (SSCD). Typical clinical and instrumental features of TMWM, including low-frequency conductive hearing loss (CHL), autophony, pulsatile tinnitus, sound/pressure-induced vertigo and enhanced vestibular-evoked myogenic potentials, have been widely described in cases with PSCD. Nevertheless, video-head impulse test (vHIT) results have been poorly investigated. Here, we present six patients with PSCD presenting with a clinical scenario consistent with a TMWM and an impaired vestibulo-ocular reflex (VOR) for the affected canal on vHIT. In two cases, an additional dehiscence between the facial nerve and the horizontal semicircular canal (HSC) was detected, leading to a concurrent VOR impairment for the HSC. While in SSCD, a VOR gain reduction could be ascribed to a spontaneous "auto-plugging" process due to a dural prolapse into the canal, the same pathomechanism is difficult to conceive in PSCD due to a different anatomical position, making a dural herniation less likely. Alternative putative pathomechanisms are discussed, including an endolymphatic flow dissipation during head impulses as already hypothesized in SSCD. The association of symptoms/signs consistent with TMWM and a reduced VOR gain for the posterior canal might address the diagnosis toward PSCD.

Acute isolated vertigo with vertical up-beating nystagmus: A rare case of nucleus intercalatus of Staderini ischemia.

The role of intercalatus nucleus of Staderini (INS), the most caudal of the perihypoglossal nuclei, is much debated. Last research seems to suggest that this nucleus plays a role as a vertical eyes movements integrator. The few clinical reports present in the literature that describe isolated lesions of the INS have described patients presenting in acute with up-beating vertical spontaneous nystagmus. Isolated acute lesion of INS is, in fact, much rare, and, without other neurological signs, is exceptional. We present a case of acute isolated vertigo with no other neurological signs or symptoms, due to INS ischemia provoked by vertebral artery stenosis. The patient presented with spontaneous vertical up-beating nystagmus that showed at videonystagmographic recording, a clear exponential decay of angular slow-phase velocity, that is considered a typical sign of neural integrator impairment. This case seems to represent a further confirm that INS is part, as a vertical-to-position neural integrator, of the neural circuit controlling the vertical eyes movements.

Paroxysmal Positional Nystagmus in Acoustic Neuroma Patients.

The association between acoustic neuroma and positional vertigo with paroxysmal positional nystagmus is relatively rare, but, when present, it certainly represents a challenge for the otoneurologist. There are few reports in the literature on this particular issue, and some questions are still unanswered, particularly regarding the characteristics of positional nystagmus that may distinguish between a true benign paroxysmal vertigo and a positional nystagmus associated with the tumor. We present the videonystagmographic patterns of seven patients with acoustic tumor who had paroxysmal positional nystagmus and analyzed its features. A concomitant true benign paroxysmal positional vertigo may be present during the follow-up of a non-treated patient, as the paroxysmal positional vertigo may be the first symptom of the tumor, and it may show characteristics that are very similar to a posterior semicircular canal canalolithiasis or a horizontal canal "heavy or light cupula". The possible mechanisms are discussed.

Increased choroidal thickness in a patient with acquired hyperopia and choroidal folds syndrome.

Purpose: syndrome of acquired hyperopia with choroidal folds is a rare syndrome characterized by flattening of the posterior pole with subsequent hyperopization of the eye and a creation of a space between the optic nerve and its sheath. Though uncommon and more often benign, it represents a diagnostic and therapeutic challenge. Correct diagnosis is helpful to exclude other possible causes of choroidal folds. Observations: here we report a case of a 39-year-old woman who presented with sudden monolateral hyperopia and choroidal folds in the affected eye. Conclusions and Importance: we performed a specific assessment of the thickness of choroid with the purpose to give further information for the understanding of the underlying condition. To date, the aspect and the thickness of the choroid in this condition, has not been evaluated yet.

Posterior semicircular canal ossification following acute vestibular loss mimicking inferior vestibular neuritis: A case report.

Vestibular neuritis (VN) mostly involves the superior vestibular nerve. Isolated inferior vestibular neuritis (IVN) has been more rarely described. The diagnosis of IVN is based on an abnormal head impulse test (HIT) for the posterior semicircular canal (PSC), pathological cervical vestibular-evoked myogenic potentials (C-VEMPs), and spontaneous downbeat nystagmus consistent with acute functional loss of inner ear sensors lying within the inferior part of the labyrinth. HIT for both lateral and superior semicircular canals is normal, as are ocular VEMPs and bithermal caloric irrigations. The etiology of IVN is debated since peripheral acute vestibular loss with a similar lesion pattern can often be associated with ipsilesional sudden hearing loss (HL). Viral inflammation of vestibular nerves is considered the most likely cause, although reports suggest that VN usually spares the inferior division. On the other hand, an ischemic lesion involving the terminal branches of the common cochlear artery has been hypothesized in cases with concurrent HL. Debated is also the lesion site in the case of IVN without HL since different instrumental patterns have been documented. Either isolated posterior ampullary nerve involvement presenting with selective PSC functional loss on video-HIT, or only saccular lesion with isolated ipsilesional C-VEMPs impairment, or inferior vestibular nerve damage (including both saccular and posterior ampullary afferents) exhibiting an impairment of both C-VEMPs and PSC-HIT. We report an interesting case of a patient with an acute vestibular loss consistent with IVN without HL who developed a PSC ossification on follow-up, questioning the viral origin of the lesion and rather orienting toward an occlusion of the posterior vestibular artery. To the best of our knowledge, this is the first report of PSC ossification after a clinical picture consistent with IVN.

Sequential Vestibular Neuritis: Report of Four Cases and Literature Review.

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.

Comparison between Fundus Automated Perimetry and Humphrey Field Analyzer: Performance and usability of the Fundus Automated Perimetry and Humphrey Field Analyzer in healthy, ocular hypertensive, and glaucomatous patients.

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 < 0.001), POAG -2.00 ± 1.66 dB (p < 0.001), and controls -1.08 ± 1.43 dB (p < 0.001). PSD's difference was higher for FAP than HFA: OHT 0.85 ± 1.16 dB (p < 0.001), POAG 0.78 ± 2.32 dB (p = 0.043), and controls 0.49 ± 1.15 dB (p < 0.001). GSS2's difference showed that FAP found more severe defects than HFA. Exams duration was longer for FAP versus HFA: in OHT 363 s versus 301 s, in POAG 494 s versus 362 s, and in controls 360 s versus 277 s. For FN and FP, there were no statistically significant differences. The 77% of all subjects preferred FAP to HFA test. 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.

Upright BPPV Protocol: Feasibility of a New Diagnostic Paradigm for Lateral Semicircular Canal Benign Paroxysmal Positional Vertigo Compared to Standard Diagnostic Maneuvers.

Background: The diagnosis of benign paroxysmal positional vertigo (BPPV) involving the lateral semicircular canal (LSC) is traditionally entrusted to the supine head roll test, also known as supine head yaw test (SHYT), which usually allows identification of the pathologic side and BPPV form (geotropic vs. apogeotropic). Nevertheless, SHYT may not always allow easy detection of the affected canal, resulting in similar responses on both sides and intense autonomic symptoms in patients with recent onset of vertigo. The newly introduced upright head roll test (UHRT) represents a diagnostic maneuver for LSC-BPPV, supplementing the already-known head pitch test (HPT) in the sitting position. The combination of these two tests should enable clinicians to determine the precise location of debris within LSC, avoiding disturbing symptoms related to supine positionings. Therefore, we proposed the upright BPPV protocol (UBP), a test battery exclusively performed in the upright position, including the evaluation of pseudo-spontaneous nystagmus (PSN), HPT and UHRT. The purpose of this multicenter study is to determine the feasibility of UBP in the diagnosis of LSC-BPPV. Methods: We retrospectively reviewed the clinical data of 134 consecutive patients diagnosed with LSC-BPPV. All of them received both UBP and the complete diagnostic protocol (CDP), including the evaluation of PSN and data resulting from HPT, UHRT, seated-supine positioning test (SSPT), and SHYT. Results: A correct diagnosis for LSC-BPPV was achieved in 95.5% of cases using exclusively the UBP, with a highly significant concordance with the CDP (p < 0.000, Cohen's kappa = 0.94), regardless of the time elapsed from symptom onset to diagnosis. The concordance between UBP and CDP was not impaired even when cases in which HPT and/or UHRT provided incomplete results were included (p < 0.000). Correct diagnosis using the supine diagnostic protocol (SDP, including SSPT + SHYT) or the sole SHYT was achieved in 85.1% of cases, with similar statistical concordance (p < 0.000) and weaker strength of relationship (Cohen's kappa = 0.80). Conclusion: UBP allows correct diagnosis in LSC-BPPV from the sitting position in most cases, sparing the patient supine positionings and related symptoms. UBP could also allow clinicians to proceed directly with repositioning maneuvers from the upright position.

Pneumolabyrinth and Recurrent Paroxysmal Positional Vertigo After Traumatic Stapes Fracture.

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.

Spontaneous Canalith Jam and Apogeotropic Horizontal Canal Benign Paroxysmal Positional Vertigo: Considerations on a Particular Case Mimicking an Acute Vestibular Deficit.

OBJECTIVE: 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. PATIENT: 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. INTERVENTIONS: 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. RESULTS: 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. CONCLUSIONS: 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.

Sneezing and Perilymphatic Fistula of the Round Window: Case Report and Systematic Review of the Literature.

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.

Longterm Safety and Efficacy of Adalimumab and Infliximab for Uveitis Associated with Juvenile Idiopathic Arthritis.

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.

Ocular nonsuicidal self-injury in a teenager.

A 14-year-old male teen presented with unilateral episcleritis, unresponsive to topical and systemic corticosteroid therapy, without a history of ocular trauma or evidence for systemic diseases. The presence of foreign bodies in the conjunctival mucus of the hyperemic fornix has been noticed during one of the follow-up examinations. The toxicological analysis of conjunctival mucus revealed the presence of ethylene glycolmonomethyl ether and triethilene glicolebuthyl ether, used as solvents in nail polish removers and all-purpose cleaners. An unexpected etiology of chemical self-inflicted episcleritis was determined. The teen was admitted to a psychological assessment, after which a psychotherapeutic treatment was recommended. Episcleritis is characterized by the acute onset of ocular pain and redness, with a frequent recurrent and stressful course. Since it can be associated with life-threatening systemic vasculitides, a prompt, aggressive immunosuppressive therapy may be considered, both for the ocular inflammation and for the underlying systemic condition. Rarely episcleritis does not improve despite topical and systemic therapy, administered in a stepladder way. The reported teenager case needed a complex multidisciplinary approach to achieve the correct diagnosis and to avoid unnecessary treatments. In the case of recognized "nonsuicidal self-injury," a psychological evaluation is strongly recommended, to identify and address underlying neuropsychiatric problems.

Ophthalmologic wax models as an educational tool for 18th-century vision scientists.

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.

Retraction pocket excision with cartilage grafting as a preventive surgery for cholesteatoma.

Aims: The goal of the present study is to summarize our experience on surgical management of retraction pockets (RP) as a preventive tool against cholesteatomas. Methods: Twenty-five ears have been followed up for a mean period of 6.16 ± 4.35 years (from 1 to 17 years). The sample presented a mean age of 47.56 ± 19.11 years (from 16 to 73 years). All patients underwent cartilage graft surgery. Furthermore 10 (40%) underwent tympanoplasty (TPL) type I, 14 (56%) TPL type II and 1 (4%) TPL type V. Results: Eleven ears (44%) showed cholesteatoma: all these cases were stage III according to Charachon staging, and stage IV or V according to Gersdorff classification. Twelve patients (48%) showed erosion of the ossicular chain. Of these, five were associated with cholesteatoma and seven only with retraction. The recurrence rate of cholesteatoma was 12%. None of the patients with a stage II or III RP (according to Gersdorff classification) developed cholesteatoma. The recurrence of RP was 0%.In regards to literature review, seven references were selected. These studies showed a success rate ranging from 79.1% to 88%, while recurrences of RP varied from 6.4% to 13%. Only one study specified a recurrence rate of cholesteatoma of 28%. Conclusions: Surgical treatment of stages II and III RP is an effective tool to prevent cholesteatoma formation. The presence of keratin accumulation and cholesteatoma at the RP (stages IV and V, according to Gersdorff) are the real predictors of poor prognosis.

Congenital Intratympanic Cholesteatoma in an Adult Patient: A Case Report and Review of the Literature.

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.

In Vivo Detection of Choroidal Abnormalities Related to NF1: Feasibility and Comparison With Standard NIH Diagnostic Criteria in Pediatric Patients.

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.