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Objective: Unusual setting of medical care Background: Acquired pendular nystagmus (APN) is a back and forth, oscillatory eye movement in which the 2 oppositely directed slow phases have similar waveforms. APN occurs commonly in multiple sclerosis and causes a disabling oscillopsia that impairs vision. Previous studies have proven that symptomatic therapy with gabapentin or memantine can reduce the nystagmus amplitude or frequency. However, the effect of these medications on visual acuity (VA) is less known and to our knowledge the impact of non-pharmacological strategies such as blinking on VA has not been reported. This is a single observational study without controls (Class IV) and is meant to suggest a future strategy for study of vision in patients with disabling nystagmus and impaired vision. Case Report: A 49-year-old woman with primary progressive multiple sclerosis with spastic paraparesis and a history of optic atrophy presented with asymmetrical binocular APN and bothersome oscillopsia. We found that in the eye with greater APN her visual acuity improved by 1 line (from 0.063 to 0.08 decimals) immediately after blinking. During treatment with memantine, her VA without blinking increased by 2 lines, from 0.063 to 0.12, but improved even more (from 0.12 to 0.16) after blinking. In the contralateral eye with a barely visible nystagmus, VA was reduced by 1 line briefly (~500 ms) after blinking. Conclusions: In a patient with APN, blinking transiently improved vision. The combination of pharmacological treatment with memantine and the blinking strategy may induce better VA and less oscillopsia than either alone. [ABSTRACT FROM AUTHOR]

DOI:10.3969/j.issn.1672⁃6731.2020.10.013

DOI:10.3969/j.issn.1672⁃6731.2020.11.016

DOI: 10.3969/j.issn.1672-6731.2019.02.011

Objective To explore the correct diagnosis and treatment of canal conversion of otolith in the treatment of geotropic nystagmus horizontal semicircular canal benign paroxysmal positional vertigo (BPPV) by Barbecue maneuver. Methods and Results There were 66 patients with geotropic nystagmus horizontal semicircular canal BPPV treated by Barbecue maneuver from January to December 2010 in Guangdong General Hospital. Among 66 cases, 6 cases (9.09%) were transformed to posterior semicircular canal BPPV and 10 cases (15.15% ) were transformed to apogeotropic nystagmus. Among 50 cases (75.76%) who did not show transition, 3 cases (4.55%) were found relapsed on the 30th day of follow-up. The total effective rate was 95.45% (63/66). No one suffered from severe adverse events. Conclusions During the maneuver treatment of geotropic nystagmus horizontal semicircular canal BPPV, canal conversion of otolith could happen. Therefore, characteristics of nystagmus should be concerned, and exact diagnosis and repeated maneuver treatment is the key to success. DOI: 10.3969/j.issn.1672-6731.2019.02.004

Objective To analyze the types and characteristics of nystagmus in patients with positional vertigo who were ineffective by particle repositioning maneuver (PRM) and had negative findings in MRI, and try to explore drug treatment and prevention programme. Methods A total of 43 patients with positional nystagmus were randomly treated with clonazepam or flunarizine hydrochloride. The therapeutic effect at acute phase was evaluated by comparing maximum slow phase velocity (SPV) before and after treatment. Among all patients, 21 patients with frequent attacks in recent 2 months were randomly divided into flunarizine hydrochloride prophylaxis treatment group (prophylaxis group) and blank control group (control group), followed up and recorded the average attack days of vertigo per month (days of single attack × number of attacks). The preventive effect of flunarizine hydrochloride was analyzed. Results Among 43 patients, 29 cases met the diagnostic criteria of definite and probable vestibular migraine. The main types of nystagmus were horizontal apogeotropic nystagmus (23 cases, 53.49% ), horizontal geotropic nystagmus (3 cases, 6.98% ), downbeat nystagmus (4 cases, 9.30% ) and mixed nystagmus (13 cases, 30.23%). Nystagmus lasted a longer time and lacked the characteristics of crescendo-decrescendo. After 3 d of treatment, the maximum SPV [2.40 (0.00, 17.15) °/s] of clonazepam group was significantly reduced, and the therapeutic effect at acute phase was better compared with flunarizine hydrochloride group [18.85 (1.58, 35.75) °/s; Z = -2.284, P = 0.022]. After 2 months, the average attack days of vertigo per month in prophylaxis group [1 (0, 2) d] was significantly reduced in comparison with control group [3.50 (1.50, 6.50) d; Z = -2.096, P = 0.036). Conclusions Positional vertigo with ineffective PRM and negative findings in MRI may be related to abnormal central function. After excluding the relevant contraindications, clonazepam can relieve vertigo and nystagmus in the acute phase, especially in vestibular migraine patients. For patients with frequent attacks, flunarizine hydrochloride maybe an available preventive treatment. DOI: 10.3969/j.issn.1672-6731.2019.02.003

Periodic alternating nystagmus (PAN) is a rare, congenital or acquired spontaneous nystagmus. Understanding the characteristics and mechanism of different types of PAN contributes to the recognition of this disease and the level of diagnosis and differential diagnosis, and helps clinicians to formulate targeted treatment. DOI: 10.3969/j.issn.1672-6731.2019.01.005

Objective To investigate the efficacy of Gufoni maneuver for treating geotropic nystagmus horizontal semicircular canal benign paroxysmal positional vertigo (BPPV). Methods We retrospectively analyzed 87 patients diagnosed as geotropic nystagmus horizontal semicircular canal BPPV from January to December 2016. All patients were treated by Gufoni maneuver twice and examined on the next day to access the status of BPPV. Patients still with geotropic nystagmus or dizziness were treated by Gufoni maneuver twice again and examined after 30 min. The effect was assessed by "Diagnosis basis and curative effect appraisal of benign paroxysmal positional vertigo (2006, Guiyang)". Results The success rate of first treatment by Gufoni maneuver achieved to 71.26% (62/87) and increased to 86.21% (75/87) after the second treatment. Among these patients, 12 patients failed to recover, 8 of whom were treated by forced prolonged position (FPP) and recovered after one week, and 4 of whom turned to be posterior semicircular canal BPPV and were treated by Epley maneuver successfully. Conclusions Gufoni maneuver is proved to be a good treatment for geotropic nystagmus horizontal semicircular canal BPPV and the efficacy increases after repeated treatment. DOI: 10.3969/j.issn.1672-6731.2019.01.008

Objective: The aim of this paper is to compare the refractive correction effects of rigid gas permeable contact lenses (RGPCL) and spectacle correction in children with aphakia after congenital cataract surgery. Methods: This was a prospective non-randomized controlled trial. Children with aphakic eyes after congenital cataract surgery, who underwent vision correction in the Strabismus and Pediatric Ophthalmology Clinic of Beijing Tongren Hospital affiliated with Capital Medical University from April 2012 to November 2019, were continuously collected. Those who voluntarily chose to wear RGPCL for refractive correction were included in the experimental group. Patients with monocular disease were in trial group 1, and patients with binocular disease were in trial group 2. Patients who chose to wear frame glasses for refractive correction were included in the control group. Patients with monocular disease were in control group 1, and patients with binocular disease were in control group 2. Regional origin, medical history, and family information were collected at the first diagnosis. During the follow-up, adverse reactions occurring during the process of wearing glasses were recorded. The Teller acuity card was used for visual examination to obtain the best-corrected visual acuity and convert it into the logarithm of the minimum resolution angle. The degree of nystagmus was determined according to the amplitude and frequency of nystagmus. Treatment cost, treatment compliance, and the reasons for adopting or not adopting RGPCL were analyzed through a questionnaire completed by the parents of children with RGPCL. Results: A total of 203 children (344 eyes) who underwent congenital cataract surgery were included, including 124 males (210 eyes) and 79 females (134 eyes). The age range was 3 to 36 months. There were 28 cases in the experimental group, including 19 cases in trial group 1 and 9 cases in trial group 2. There were 175 cases in the control group, including 43 cases in control group 1 and 132 cases in control group 2. Except for 6 months of age, the visual acuity of the experimental group was better than that of the control group, and the differences were statistically significant ( P <0.05). The visual acuity of children in trial group 1 was better than that of children in control group 1 at the same age. Among them, at 12 months of age [1.54 (1.27, 1.97), 1.84 (0.97, 2.12)], 18 months of age [1.27 (0.97, 1.84), 1.84 (0.97, 2.12)], 24 months of age [1.54 (1.27, 1.84), 1.84 (0.97, 2.12)], and 30 months old [0.97 (0.66, 1.27), 1.54 (0.66, 2.12)], the difference was statistically significant ( P <0.001). The visual acuity of children in trial group 2 was better than that in control group 2 at the same age. Among them, at 18 months old [1.27 (0.97, 1.54), 1.27 (0.66, 2.12)], 24 months old [0.97 (0.66, 1.27), 1.27 (0.66, 2.12)], and 30 months old [1.27 (0.66, 2.12)], the difference was statistically significant ( P <0.05). The remission rate of nystagmus in the experimental group was 8/9 (8 cases), the remission rate of nystagmus in the control group was 34.40% (32 cases), and the exacerbation rate was 29.03% (27 cases). The average annual cost of the experimental group was 25 125 yuan, and that of the control group was 2 511 yuan. Conclusions: RGPCL is a well-tolerated, safe, and effective treatment for infants and young children. The visual acuity and degree of nystagmus were significantly improved in children who wore RGPCL for aphakia refractive correction after congenital cataract surgery compared with spectacle correction.

Posterior fossa tumors (PFT) are the most common pediatric brain tumors, and the study of the somatic and cognitive status of PFT survivors still remains a critical problem. Since cerebellar damage can affect eye movement centers located in the vermis and hemispheres, such patients suffer from disturbances in visual perception, visual-spatial functions, reading, etc. Our investigation aimed at describing oculomotor impairments in PFT survivors linked to core oculomotor functions assessed through eye tracking method: gaze holding, reflexive saccades, and organization of voluntary saccades and their dependency on age at tumor diagnosis. Also, we investigated the relationship between oculomotor functions and ataxia measured with International Cooperative Ataxia Rating Scale (ICARS). A total of 110 children (patients and age-matched healthy controls, aged 9-17 years old) participated in the study. We found that the earlier the child had a tumor, the more impaired gaze holding (p = 0.0031) and fewer isometric saccades (p = 0.035) were observed at the time of examination. The above-mentioned functions in healthy controls improved with age. Visual scanning was also impaired compared to controls but was not related to age at diagnosis. A positive correlation between ICARS scores and number of hypermetric saccades (r = 0.309, p = 0.039), but no correlation with the number of hypometric saccades (r = - 0.008, p = 0.956). Furthermore, number of hypometric saccades did not differ between patients and controls (p = 0.238). Thus, primarily hypermetric saccades can be considered a prominent oculomotor symptom of cerebellar tumors. Our study provides basis for new methods of PFT diagnosis and rehabilitation procedure evaluation, both playing essential roles in modern pediatric neurooncology., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Objectives: To evaluate the therapeutic efficacy of the forced prolonged position (FPP) in patients with horizontal semicircular canal (HSCC) cupulolithiasis in whom the cupulolith repositioning maneuver (CuRM) failed., Methods: Fifty-four consecutive patients with HSCC cupulolithiasis were included, and immediate treatment efficacy of CuRM and short-term treatment efficacy of FPP were investigated., Results: We performed the CuRM in HSCC cupulolithiasis, and, if the CuRM did not show immediate success, instructed the patients to perform the FPP or the sham position (by random allocation) at home. The immediate therapeutic success of the CuRM was assessed by the absence of nystagmus and vertigo on positional testing after 30 minutes of the maneuver, which was 14.8% on the first visit day. And the resolution rate of HSCC cupulolithiasis was higher in the FPP group than in the sham position group at the second (78.3% vs 55.6%), third (75.0% vs 42.9%), and fourth visits (100% vs 25.0%)., Conclusion: Although the CuRM has been considered to be a good therapeutic option for HSCC cupulolithiasis because it theoretically aims to detach otoconial particles attached both on the utricle and canal sides of the cupula, the immediate success rate was only 14.8% at the first visit. The FPP can be additionally recommended to improve the resolution rate in HSCC cupulolithiasis patients in whom the CuRM fails., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

After unilateral peripheral vestibular lesions, the neural activity of neurons in the ipsi-lesional medial vestibular nucleus (ipsi-MVe) are markedly decreased, resulting in static and dynamic asymmetries of the vestibulo-ocular and vestibulo-spinal reflexes. Consequently, static vestibular symptoms such as spontaneous nystagmus and postural deviation and dynamic vestibular symptoms such as oscillopsia and swaying gait are induced. However, these behavioral asymmetries gradually recover after the lesion. Progressive balance restoration is termed vestibular compensation, which is divided into two phases: static and dynamic. Static vestibular compensation is further divided into initial and late processes. In the initial process of static vestibular compensation after unilateral labyrinthectomy (UL) in rats, plastic changes in the cerebello-vestibular and vestibular commissural inhibitory pathways suppress neurons in the contra-lesional MVe (contra-MVe), resulting in the restoration of symmetrical resting activity of MVe neurons on both sides at low levels. The declining frequency of spontaneous nystagmus after UL is an index of the initial process, and short-term administration of diazepam, a GABA A receptor agonist, has been shown to accelerate the initial process in rats. Accordingly, short-term administration of diazepam is recommended for the treatment of acute vertigo in patients with unilateral vestibular dysfunction. In the late process of static vestibular compensation after UL in rats, the resting activity of ipsi-MVe neurons gradually recovers due to changes in cell membrane properties, resulting in the reinforcement of balanced intervestibular nuclear activities to nearly normal levels without the suppression of contra-MVe neurons. The declining number of MK801-induced Fos-positive neurons in contra-MVe after UL is an index of the late process, and long-term administration of betahistine, a histamine H 3 receptor antagonist, has been shown to accelerate the late process in rats. Accordingly, long-term administration of betahistine is recommended for the treatment of subacute vertigo in patients who were not compensated for unilateral vestibular dysfunction. In the process of dynamic vestibular compensation after UL, the sensitivity of ipsi-MVe neurons to head velocity and acceleration is restored due to synaptic changes such as long-term potentiation and sprouting of commissures, resulting in the restoration of the dynamic vestibulo-ocular and vestibulo-spinal reflexes. To facilitate dynamic vestibular compensation, early ambulation and subsequent vestibular rehabilitation exercise are recommended for the treatment of chronic vertigo in patients with uncompensated unilateral vestibular dysfunction. Although vestibular compensation after bilateral vestibular loss is not expected, vestibular rehabilitation with a sensory-substitution strategy can improve imbalance in patients with bilateral vestibular lesions., Competing Interests: Declaration of Competing Interest The authors declare no conflicts of interest in this review., (Copyright © 2023. Published by Elsevier B.V.)

The purpose of this paper is to expand on the phenotype of oculocutaneous albinism type 7 (OCA7). We described three patients with OCA7: two from a consanguineous family of Kurdish origin and one patient of Dutch origin. We compared them with all patients described to date in the literature. All newly described patients had severely reduced visual acuity (VA), nystagmus, hypopigmentation of the fundus, severe foveal hypoplasia, and chiasmal misrouting. None had iris translucency. All patients had normal pigmentation of skin and hair. We found one novel mutation in the Dutch patient: c.565G > A; p.(Gly189Ser). We compared our patients to the 15 described in the literature to date. All 18 patients had substantially pigmented skin and hair, very poor VA (0.4-1.3 logMAR), nystagmus, (mild) ocular hypopigmentation, foveal hypoplasia, and misrouting. Although pigmentation levels were mildly affected in OCA7, patients had a severe ocular phenotype with VA at the poorer end of the albinism spectrum, severe foveal hypoplasia, and chiasmal misrouting. OCA7 patients had a phenotype restricted to the eyes, and similar to that of X-linked ocular albinism. We therefore propose to rename the disorder in ocular albinism type 2. Unfolding the role of LRMDA in OCA7, may bring us a step closer in identifying the responsible factors for the co-occurrence of foveal hypoplasia and misrouting., (© 2024. The Author(s).)

Competing Interests: The authors report no conflicts of interest.

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Objective: To study the relationships of functional and morphologic retinal parameters in a series of pediatric patients with varying degrees of foveal hypoplasia (FH)., Design: Monocentric observational retrospective study., Participants: Among 21 pediatric patients, 16 met inclusion criteria, having FH confirmed with spectral-domain optical coherence tomography (SD-OCT) scan METHODS: Data were analyzed retrospectively. Patients able to undergo macular microperimetry (MP) and SD-OCT examinations were included in the analysis. MP and SD-OCT outcomes were compared with FH grading and best-corrected visual acuity (BCVA) using Pearson's correlation., Results: Thirty-one eyes from 16 patients (mean age 12.4 years) with different degrees of FH were analyzed. Two patients had grade 1, 7 had grade 2, 5 had grade 3, and 2 had grade 4 FH. Clinical nystagmus was present in 8 patients. The correlation between BCVA and SD-OCT data (-0.31) was lower than that found between BCVA and nystagmus (0.64), that for fixation index P1 (-0.60), as well as that for macular sensitivity (-0.63)., Conclusions: Although limited by the small sample, our study confirms the feasibility of automated MP evaluation in pediatric patients with FH. The added value of this work is the provision of data on relationships between anatomic and functional macular measurements acquired with SD-OCT, MP, and BCVA in eyes with various degrees of FH. Larger prospective studies are necessary to confirm these results., (Copyright © 2022 Canadian Ophthalmological Society. Published by Elsevier Inc. All rights reserved.)

Background: Nystagmus generated during bithermal caloric test assesses the horizontal vestibulo-ocular-reflex. Any induced symptoms are considered unwanted side effects rather than diagnostic information., Aim: We hypothesized that nystagmus slow-phase-velocity (SPV) and subjective symptoms during caloric testing would be higher in vestibular migraine (VM) patients compared with peripheral disorders such as Meniere's disease (MD) and non-vestibular dizziness (NVD)., Methods: Consecutive patients (n = 1373, 60% female) referred for caloric testing were recruited. During caloric irrigations, patients scored their subjective sensations. We assessed objective-measures, subjective vertigo (SVS), subjective nausea (SNS), and test completion status., Results: Nystagmus SPV for VM, MD (unaffected side), and NVD were 29 ± 12.8, 30 ± 15.4, and 28 ± 14.2 for warm irrigation and 24 ± 8.9, 22 ± 10.0, and 25 ± 12.8 for cold-irrigation. The mean SVS were 2.5 ± 1.1, 1.5 ± 1.33, and 1.5 ± 1.42 for warm irrigation and 2.2 ± 1.1, 1.1 ± 1.19, and 1.1 ± 1.16 for cold-irrigation. Age was significantly correlated with SVS and SNS, (p < 0.001) for both. The SVS and SNS were significantly higher in VM compared with non-VM groups (p < 0.001), and there was no difference in nystagmus SPV. VM patients SVS was significantly different to the SVS of migraineurs in the other diagnostic groups (p < 0.001). Testing was incomplete for 34.4% of VM and 3.2% of MD patients. To separate VM from MD, we computed a composite value representing the caloric data, with 83% sensitivity and 71% specificity. Application of machine learning to these metrics plus patient demographics yielded better separation (96% sensitivity and 85% specificity)., Conclusion: Perceptual differences between VM and non-VM patients during caloric stimulation indicate that subjective ratings during caloric testing are meaningful measures. Combining objective and subjective measures could provide optimal separation of VM from MD., (© 2023. The Author(s).)

Competing Interests: Competing interests: None declared.

Purpose: To determine if visual maturation continues beyond the first decade of life in children with albinism and whether this is related to albinism type, presence of nystagmus, eye muscle surgery or refractive errors., Design: Case series based on retrospective study of children with confirmed genetic diagnosis of albinism., Methods: Clinical data were obtained from medical files of children examined during school years, including albinism type, visual acuity, eye muscle surgery, nystagmus, and others on different visits (Visit 1: ages 7-9; Visit 2: ages: 10-12; Visit 3: ages 13-16; Visit 4: ages >16)., Results: Seventy-five children with albinism were included in the study. Patients were divided into different groups according to the albinism type including OCA1A: 17; OCA1B: 28; OCA2: 26; HPS: 3; OCA4: 1. Follow-up ranged from 3-13 years. Progressive visual acuity improvement was seen in all three main groups. T-test paired samples showed a statistically significant improvement when comparing vision from Visit 1 and Visit 3 in both OCA1A and OCA2 groups, with a mean vision improvement of 2 lines. There was no correlation between visual improvement and refractive error, eye muscle surgery or nystagmus., Conclusion: An improved visual performance was seen in a large percentage of children with albinism during the second decade of life. The reason for this late improvement in vision is not clear but may be related to late foveal maturation or improvement in nystagmus with time. This information is useful for clinicians of these patients and when counseling parents., Competing Interests: The authors have declared that no competing interests exist, (Copyright: © 2024 Yahalom et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.)