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Functional outcome after pyeloplasty in children: impact of the cause of obstruction and of the mode of presentation.

OBJECTIVE: To compare functional outcome after pyeloplasty in two groups of patients affected by hydronephrosis, which had different modalities of diagnosis. The first, asymptomatic, detected by prenatal ultrasonography and the second referred later because of clinical symptoms. PATIENTS AND METHODS: 84 patients (54 prenatally detected and 30 symptomatic) among 339 observed with hydronephrosis, operated in a single centre for ureteropelvic junction obstruction, have been retrospectively studied. Seventeen cases with prenatal diagnosis had an early treatment and thirty-seven were operated on after an initial observation. Symptomatic cases were all operated on at diagnosis. Ultrasonographic and renographic assessment were made at diagnosis, before and after surgery. RESULTS: Renal function of prenatally detected cases was not influenced by early or delayed surgery. Insignificant functional loss was recorded among some cases operated after conservative management. Improvement in differential renal function (p<0.05) was recorded in symptomatic patients after pyeloplasty. A high percentage of obstructions secondary to lower polar crossing vessel were found among symptomatic cases (12 out of 30). Intrinsic obstructions were predominant among cases with in utero diagnosis (48 out of 54). Whenever the cause of obstruction is considered, postoperative functional improvement was limited to crossing vessels cases. CONCLUSIONS: Renal function among antenatally diagnosed obstructions is scarcely influenced by surgery; potential risk of further renal damage cannot be excluded when expectant management is decided. Later detected, symptomatic cases show a better functional response to surgery. This seems corresponding to distinct clinical entities based on different causes of hydronephrosis. A reversible renal damage seems to be associated to extrinsic obstructions from polar vessel, which are predominant among symptomatic, later detected cases. A congenital, irreversible loss of function accompanies intrinsic obstructions, typical of prenatally diagnosed cases.

Child↗

Neuro-otological findings after radiosurgery for acoustic neurinomas.

OBJECTIVE: To evaluate the neuro-otological complications in patients after radiosurgery for acoustic neurinomas. DESIGN: Inception cohort, retrospective study. SETTING: University hospital. PATIENTS: A consecutive sample of 46 patients with acoustic neurinomas who underwent unilateral gamma knife radiosurgery at the University of Tokyo, Japan, between June 1990 and June 1994 were followed up by otolaryngologists for more than 3 months. INTERVENTION: Gamma knife stereotactic radiosurgery. MAIN OUTCOME MEASURES: Neuro-otological examinations including pure tone audiometry, auditory brain stem response, and caloric test. RESULTS: Tumor growth occurred in 2 patients (4.3%). Seven (18%) of the 38 patients with preserved hearing of any extent became deaf within 1 year. In cases of gradual hearing loss, the average deterioration rate was approximately 8 dB per year. Abnormalities of auditory brain stem response preceded deafness in 2 patients. Caloric response, preserved before treatment in 13 patients, disappeared 4 to 13 months after treatment (median, 8 months) in 9 (69%) of them, whereas their hearing was preserved. Delayed facial palsy and persistent trigeminal neuropathy occurred in 10 (22%) and 7 (15%) of the 46 patients, respectively. Severe facial palsy tended to persist. CONCLUSIONS: The rates of neuro-otological complications of radiosurgery are almost comparable with those previously reported from other institutions. The deafness within 1 year after treatment might be attributed to a lesion in the cochlear nerve. Hearing loss did not parallel vestibular function loss. The persistent severe facial palsy contrasts with previously reported findings. Considering the serious facial nerve complications that occurred in some of our patients, further study to disclose the risk factors for neurological dysfunction would be needed for radiosurgery to become a true, safe alternative to microsurgery.

Adolescent↗

Reliability of island flaps raised after superficial and deep burn injury.

In select cases, to prevent any functional loss and to initiate early function during the early burn period, the reconstructive procedure of choice may be flap coverage. In these circumstances, when the ideal flap donor site is burned, the clinician may be hesitant to raise this flap because of questionable flap survival. The authors conducted this study to determine whether a superficially or deeply burned skin island flap would survive when elevated during the early postburn period. If these flaps are usable, they could expand the options available for burn wound coverage. They used a rat epigastric island flap model, and divided 50 study animals into two groups. In group 1 (N = 25), the right epigastric flap site was burned superficially and the left side was left uninjured. Island flaps were raised on both sides 4 days after the burn injury. The flaps were then sutured back into their original sites, and were evaluated 5 days after the surgery. In group 2 (N = 25), the right epigastric flap site was burned deeply and the left side was left uninjured. Island flaps were raised 4 days after the burn injury on both sides, as in group 1. The flaps were then sutured back into place and were evaluated 5 days after the surgery. All of the control flaps on the rats' left sides survived in both groups. In addition, all the superficially burned flaps survived in group 1 (100%), and 21 of the deeply burned flaps survived in group 2 (84%). There was no significant difference between superficially and deeply burned flaps with regard to survival, and the burned flaps were as successful as the unburned control flaps in both groups (p = 0.11). Skin island flaps elevated after superficial or deep burn injury are reliable in this animal model.

Animals↗

Midline sagittal sacral fractures in anterior-posterior compression pelvic ring injuries.

OBJECTIVE: To evaluate the outcome of an uncommon variant of the anterior-posterior compression pelvic injury, in which the posterior ring injury is a midline sagittal sacral fracture extending into the spinal canal. DESIGN: Prospective, consecutive series. SETTING Two regional trauma centers. PATIENTS: A consecutive series of 10 patients with rotationally displaced, vertically stable anterior-posterior compression pelvic ring fractures (OTA type 61-B1) in which the posterior ring injury is a midline sagittally oriented sacral fracture involving the spinal canal (Denis zone III). This injury pattern comprised 0.6% of pelvic fractures and 1.4% of sacral fractures treated at these two institutions during a 10-year period. INTERVENTION: Patients were treated according to the same principles used in more commonly seen types of anterior-posterior compression pelvic ring injuries. Nine patients were treated with reduction and anterior pelvic stabilization at an average of 5 days after injury, 8 of whom were treated with open reduction and internal fixation and 1 with external fixation. No posterior pelvic fixation was used. One patient with nondisplaced bilateral pubic ramus fractures was treated nonoperatively. Immediate weight bearing was allowed as tolerated. MAIN OUTCOME MEASUREMENTS: Prospectively collected clinical follow-up data emphasized a detailed neurologic examination, whereas radiographic evaluation involved anteroposterior, inlet, and outlet plain radiographic views of the pelvis. RESULTS: An anatomical or near-anatomical reduction of the pelvis was achieved and maintained in all patients. Fractures healed at an average of 10 weeks. At an average follow-up of 31 months (range 20-46 months), there were no objective neurologic deficits that could be attributed to sacral root injury and no significant residual pain or gait disturbance related to the pelvic fracture. Loss of bowel or bladder function, loss of perianal sensation or sphincter tone, and lumbosacral radicular pain or sensorimotor deficit were specifically absent in all patients. Three patients, however, complained of sexual dysfunction at final follow-up. None of these patients had clinical evidence of sacral root/plexus injury secondary to the fracture. One additional patient, who sustained a urethral tear, required a chronic suprapubic catheter because of stricture. Six patients, one of whom had needed repair of a retroperitoneal bladder tear, had no urogenital sequelae. DISCUSSION AND CONCLUSION: Patients who sustain sagittally oriented midline fractures of the sacrum that extend into the spinal canal (Denis zone III) as part of displaced, vertically stable anterior-posterior compression pelvic injuries, have a low incidence of neurologic deficit attributable to sacral root or plexus injury. This is in contrast to the high rate of neurologic deficit (>50%) otherwise reported in zone III sacral fractures, particularly in those associated with a displaced transverse component. In the midline sagittal fracture variant, simultaneous lateral displacement of both bony and neural elements through the midline may protect the sacral roots and plexi from significant traction or shear injury by maintaining the spatial orientation between the sacral foramina and sciatic notch. Long-term sequelae were related to urogenital complaints rather than to musculoskeletal problems, as 4 of the 10 patients in this series had either sexual or urologic dysfunction.

Adult↗

A case of insertional translocation involving chromosomes 2 and 4.

We report on a 6-year-old Caucasian boy with direct insertion of genetic material from the short arm of chromosome 4 to the short arm of chromosome 2. He was referred for evaluation because of global developmental delay and seizure disorder. A karyotype performed at 4 1/2 months of age, by a laboratory elsewhere, reportedly showed a deletion of chromosome 4(p12). When we saw him, he had macrocephaly, hypotonia, psychomotor retardation, multiple minor congenital anomalies, and EEG abnormalities. Repeat chromosomes performed by our laboratory revealed that his karyotype was 46,XY,dir ins(2;4)(p24;p15.3p13). Fluorescence in situ hybridization (FISH) analysis, using chromosomes 2 and 4 painting probes confirmed that material from 4p has been translocated to 2p. Also, FISH analysis using the Wolf-Hirschhorn critical region probe revealed that both loci are intact. Parental chromosomes were normal. This complex rearrangement, though it appears balanced, probably might have resulted in either a structural loss of genetic material or functional loss of a gene action. Thus, his phenotype could be explained by this de novo insertion of chromosome 4 material into chromosome 2. There is no reported case of this specific chromosome rearrangement.

Child↗

Searching for stem cell regulatory molecules. Some general thoughts and possible approaches.

Hematopoietic development in the mammal can be represented as a numerically expanding hierarchy of cell populations that are progressively restricted in their self-renewal and differentiation abilities. Classical functional studies have now been extended to provide exact physical descriptions of various stages in the hematopoietic hierarchy. In particular, much information is available that defines the properties of the most primitive stem cell compartment. In addition, a number of in vitro culture systems suggest the possibility of maintaining and expanding these cells in a defined context. In all developmental systems, unique profiles of expressed genes define distinct differentiation stages. Within these profiles are gene products that play crucial roles in the regulation of cell-fate decisions. Recent progress in hematopoietic biology provides the framework within which to define molecular phenotypes for hematopoietic stem cells and their immediate clonal progeny. Identifying novel gene products expressed predominantly in uncommitted stem cells together with functional loss and gain-of-function approaches should begin to unravel the molecular mechanisms that govern biological phenomena such as self-renewal, commitment, and proliferation in the hematopoietic system.

Animals↗

[Ophthalmology].

UNLABELLED: The present article describes the eye injuries observed in victims of the terrorist attack of 11 March in our hospital. Of the 325 injured individuals who arrived from the sites of explosion, 47 (15%) required specialized ophthalmologic care. Of these 41 were treated on 11 March and the 6 remaining patients were treated on subsequent days. In 5 patients emergency interventions were required. In 3 patients, interventions were delayed. RESULTS: anatomical loss in 1 patient and functional loss in 3 patients.

Adolescent↗

Perifoveal function in patients with North Carolina macular dystrophy: the importance of accounting for fixation locus.

PURPOSE: To quantify the extent of visual function losses in patients with North Carolina Macular Dystrophy (NCMD) and to demonstrate the importance of accounting for eccentric fixation when making comparisons with normal data. METHODS: Five patients with NCMD who were from a single family were examined. Multifocal electroretinograms (mfERGs) and psychophysical assessments of acuity and luminance visual field sensitivities were measured throughout the central retina. Comparisons of responses from equivalent retinal areas were accomplished by shifting normal templates to be centered at the locus of fixation for each patient. RESULTS: Losses of psychophysically measured visual function in patients with NCMD extend to areas adjacent to the locations of visible lesions. The multifocal ERG amplitude was reduced only within the area of visible lesion. Multifocal ERG implicit times were delayed throughout the entire central retinal area assessed. CONCLUSIONS: ERG timing is a sensitive assay of retinal function, and our results indicate that NCMD has a widespread effect at the level of the mid and outer retina. The findings also demonstrated that it is necessary to account for fixation locus and to ensure that equivalent retinal areas are compared when testing patients with macular disease who have eccentric fixation.

Adult↗

Loss of heterozygosity analyzed by single nucleotide polymorphism array in cancer.

Neoplastic progression is generally characterized by the accumulation of multiple genetic alterations including loss of tumor suppression gene function. Loss of heterozygosity (LOH) has been used to identify genomic regions that harbor tumor suppressor genes and to characterize different tumor types, pathological stages and progression. LOH pattern has been detected by allelotyping using restriction fragment length polymorphism, and later by simple sequence length polymorphisms (SSLPs or microsatellite) for 10 years. This paper reviews the detection of LOH by recently developed single nucleotide polymorphism (SNP) arrays (all analyzed by Affymetrix array); furthermore, its advantage and disadvantage were analyzed in several kinds of cancer.

Genes, Tumor Suppressor↗

Mechanical properties of the lung in diffuse interstitial lung disease.

To define the mechanical properties of the lungs in diffuse interstitial lung disease (DILD) the static deflation volume-pressure (VP) and maximum expiratory flow-volume characteristics were measured in 18 patients with DILD including 14 with biopsy confirmation. Elastic recoil, defined by the position of the VP curve, was increased in 12, normal in four and decreased in two patients. The distensibility of inflatable lung, defined by the constant K of a single exponential fitted to the VP data, was decreased in 10 of the 12 with increased recoil and increased in the two with decreased recoil. Those with a normal or increased pulmonary distensibility smoked more than those with decreased distensibility (p less than 0.05). Maximum expiratory flow rates (MEFR) were decreased at any elastic recoil pressure in 12 patients; when corrected for lung size MEFR were decreased in seven patients, six of whom were smokers. These results show that in DILD a decreased pulmonary distensibility is common but not invariable, that an increased elastic recoil reflects both loss of lung units and decreased distensibility of inflatable units and that an increased flow resistance is common. Co-existent emphysema could explain a normal or increased pulmonary distensibility in DILD. The increased flow resistance is due in part to fibrosis and loss of lung units with functional loss of the attendant airways. Decreased MEFR, despite correction for lung size, in seven subjects suggests that structural narrowing of airways, due to cigarette smoking and/or bronchiolar involvement by the disease, also contributes to an increased flow resistance in some patients with DILD.

Airway Resistance↗

Allelotyping of all chromosomal arms in invasive cervical cancer.

The best characterized factor in the development of cervical cancer is the integration, of human papillomavirus into cervical cell chromosomes. In addition to HPV integration, the neoplastic process probably requires the activation of cellular protooncogenes and loss of tumor suppressor gene function. Loss of heterozygosity analysis in a large sample is used to identify regions which harbor putative tumor suppressor genes (TSG) since the deletion of normal alleles unmask mutated alleles. We evaluated tumor tissue from invasive cervical carcinomas, carefully microdissected to eliminate normal stroma and lymphocytes, for LOH at all 41 chromosomal arms with 50 polymorphic markers. We have evaluated tumor and normal DNA pairs from 48 invasive cervical cancers of which 85% of the tumors are confined to the cervix. The mean loss for all chromosomal arms was 12%. Three regions exhibited LOH two standard deviations above the mean: 3p14.1-12 (40%), 11q23.3 (36%), and 6p22-21.3 (32%). Three regions showed loss one standard deviation above the mean: 19q13.4 (30%), 6q21-23.33 (25%), and 2q33-37 (24%). Our results indicate that a significant number of invasive cervical cancers have lost specific chromsomal regions, thereby suggesting that genes involved in the cell cycle regulation or the suppression of tumor development are located in these regions.

Alleles↗

Effector genes of Xanthomonas axonopodis pv. vesicatoria promote transmission and enhance other fitness traits in the field.

Establishing durable disease resistance in agricultural crops, where much of the plant defense is provided through effector-R gene interactions, is complicated by the ability of pathogens to overcome R gene resistance by losing the corresponding effector gene. Many proposed methods to maintain disease resistance in the field depend on the idea that effector gene loss results in a fitness cost to the pathogen. In this article we test for fitness costs of effector gene function loss. We created directed knockouts of up to four effector genes from the bacterial plant pathogen Xanthomonas axonopodis pv. vesicatoria (Xav) and examined the effect of the loss of a functional gene product on several important fitness parameters in the field. These traits included transmission, lesion development, and epiphytic survival. We found that the products of all four effector genes had significant and often additive effects on fitness traits. Additional greenhouse tests revealed costs of effector gene loss on in planta growth and further showed that the effects on lesion development were separable from the effects on growth. Observable fitness effects of the three plasmid-borne effector genes were dependent upon the loss of functional avrBs2, indicating that complex functional interactions exist among effector genes with Xav.

Analysis of Variance↗

Experimental analyses of the function of the proepicardium using a new microsurgical procedure to induce loss-of-proepicardial-function in chick embryos.

The proepicardium (PE) is a primarily extracardiac progenitor cell population that colonizes the embryonic heart and delivers the epicardium, the subepicardial and intramyocardial fibroblasts, and the coronary vessels. Recent data show that PE-derived cells additionally play important regulatory roles in myocardial development and possibly in the normal morphogenesis of the heart. Developmental Dynamics 233, 2005. Research on the latter topics profits from the fact that loss-of-PE-function can be experimentally induced in chick embryos. So far, two microsurgical techniques were used to produce such embryos: (1) blocking of PE cell transfer with pieces of the eggshell membrane, and (2) mechanical excision of PE. Both of these techniques, however, have their shortcomings. We have searched, therefore, for new techniques to eliminate the PE. Here, we show that loss-of-PE-function can be induced by photoablation of the PE. Chick embryos were treated in ovo by means of a window in the eggshell at Hamburger and Hamilton (HH) stage 16 (iday 3). The pericardial coelom was opened, and the PE was externally stained with a 1% solution of Rose Bengal by means of a micropipette. Photoactivation of the dye was accomplished by illumination of the operation field with visible light. Examination on postoperative day 1 (iday 4, HH stages 19/20) disclosed complete removal of PE in every experimental embryo. On iday 9 (HH stages 33/34), the survival rate of experimental embryos was 35.7% (15 of 42). Development of the PE-derivatives was compromised in the heart of every survivor. The abnormalities encompassed hydro- or hemopericardium, epicardium-free areas with aneurysmatic outward bulging of the ventricular wall, thin myocardium, defects of the coronary vasculature, and abnormal tissue bridges between the ventricles and the pericardial wall. Our results show that photoablation of the PE is a powerful technique to induce long-lasting loss-of-PE-function in chick embryos. We have additionally obtained new data that suggest that the embryonic epicardium may make important contributions to the passive mechanics of the developing heart.

Animals↗

Pararenal aortic aneurysms: the future of open aortic aneurysm repair.

PURPOSE: As endovascular stent graft repair of infrarenal abdominal aortic aneurysms (AAAs) becomes more common, an increasing proportion of patients who undergo open operation will have juxtarenal aneurysms (JR-AAAs), which necessitate suprarenal crossclamping, suprarenal aneurysms (SR-AAAs), which necessitate renal artery reconstruction, or aneurysms with associated renal artery occlusive disease (RAOD), which necessitate repair. To determine the current results of the standard operative treatment of these patterns of pararenal aortic aneurysms, we reviewed the outcome of 257 consecutive patients who underwent operation for JR-AAAs (n = 122), SR-AAAs (n = 58), or RAOD (n = 77). METHODS: The patients with SR-AAAs and RAOD were younger (67.5 +/- 8.8 years) than were the patients with JR-AAAs (70.5 +/- 8.3 years), and more patients with RAOD were women (43% vs 21% for JR-AAAs and SR-AAAs). The patient groups were similar in the frequency of coronary artery and pulmonary disease and in most risk factors for atherosclerosis, except hypertension, which was more common in the RAOD group. Significantly more patients with RAOD had reduced renal function before surgery (51% vs 23%). Supravisceral aortic crossclamping (above the superior mesenteric artery or the celiac artery) was needed more often in patients with SR-AAAs (52% vs 39% for RAOD and 17% for JR-AAAs). Seventeen patients (7%) had undergone a prior aortic reconstruction. The most common renal reconstruction for SR-AAA was reimplantation (n = 37; 64%) or bypass grafting (n = 12; 21%) and for RAOD was transaortic renal endarterectomy (n = 71; 92%). Mean AAA diameter was 6.7 +/- 2.1 cm and was larger in the JR-AAA (7.1 +/- 2.1 cm) and SR-AAA (6.9 +/- 2.1 cm) groups as compared with the RAOD group (5.9 +/- 1.7 cm). RESULTS: The overall mortality rate was 5.8% (n = 15) and was the same for all the groups. The mortality rate correlated (P <.05) with hematologic complications (bleeding) and postoperative visceral ischemia or infarction but not with aneurysm group or cardiac, pulmonary, or renal complications. Renal ischemia duration averaged 31.6 +/- 21.6 minutes and was longer in the SR-AAA group (43.6 +/- 38.9 minutes). Some postoperative renal function loss occurred in 104 patients (40.5%), of whom 18 (7.0%) required dialysis. At discharge or death, 24 patients (9.3%) still had no improvement in renal function and 11 of those patients (4.3%) remained on dialysis. Postoperative loss of renal function correlated (P <.05) with preoperative abnormal renal function and duration of renal ischemia but not with aneurysm type, crossclamp level, or type of renal reconstruction. CONCLUSION: These results showed that pararenal AAA repair can be performed safely and effectively. The outcomes for all three aneurysm types were similar, but there was an increased risk of loss of renal function when preoperative renal function was abnormal. These data provide a benchmark for expected treatment outcomes in patients with these patterns of pararenal aortic aneurysmal disease that currently can only be managed with open repair.

Aged↗

Factors affecting the loss of mitochondrial function in autolyzing cardiac muscle.

Rates of loss of mitochondrial respiratory function were monitored during autolyses of canine myocardial samples pretreated so as to affect tissue pH and/or tissue ATP content prevailing during tissue autolysis. When autolyses occurred under conditions of differing tissue pH, but at nearly identical tissue ATP levels, the rate of loss of mitochondrial function was virtually unchanged suggesting that tissue acidosis in the absence of a concomitant tissue ATP differential had little or no effect upon the rate of progression of mitochondrial damage. In a second comparison, autolyses were carried out at constant tissue pH, but where tissue ATP content differed dramatically. Here, the rate of loss of mitochondrial function was increased markedly suggesting that tissue ATP depletion in the absence of a concomitant tissue pH differential had a major effect upon the rate of loss of mitochondrial function. Thus, of the two parameters studied, tissue ATP content alone was far more important than tissue pH alone in determining the rate of cell membrane damage during ischemia. Finally, autolyses were carried out where both tissue pH and ATP content differed. Here, an even more dramatic increase in the rate of progression of mitochondrial damage occurred suggesting the operation of synergism between tissue ATP depletion and acidosis in promoting cell injury in ischemic cardiac muscle.

Acidosis↗

Polymorphonuclear neutrophils in posttraumatic osteomyelitis: cells recovered from the inflamed site lack chemotactic activity but generate superoxides.

The pathogenesis of posttraumatic osteomyelitis, one of the major complications after orthopedic surgery, is not yet understood. Formation of bacterial biofilms on the implant is presumed, conferring resistance to antibiotic therapy and probably also to the host defense mechanisms. In that context, the polymorphonuclear neutrophils (PMN) having infiltrated the infected site were recovered and characterized phenotypically and functionally. Loss of CD62L and upregulation of CD14 were seen, as was expression of CD83. Expression of the latter is dependent on de novo protein synthesis and thus is indicative of an extended life span and a transdifferentiation of the PMN at the infected site. The infiltrated PMN had lost their chemotactic activity, whereas the capacity to produce superoxides was preserved and in some patients even enhanced. In vitro experiments done in parallel showed that long-term culture with interferon-gamma resulted in similar alterations of PMN: loss of chemotactic activity, whereas other functions of PMN, such generation of superoxides and phagocytosis of opsonized bacteria, were preserved or even enhanced. The loss of the migratory capacity of PMN having already emigrated from the blood vessel to the infected site is not expected to affect the host defense negatively. Assuming, however, that bacteria are organized as a biofilm and that infiltration into this biofilm is required for phagocytosis of the bacteria, our data could to some extent explain why despite being activated, the PMN are not able to control the infection. By releasing their cytotoxic, proteolytic, and collagenolytic potential, PMN might instead contribute to tissue destruction and eventually to osteolysis.

Adult↗

Bilateral loss of vestibular function: clinical findings in 53 patients.

The clinical presentations and aetiologies of a series of 53 cases of bilateral vestibular failure (BVF) seen by the authors over a decade were evaluated by retrospective review of the medical records. Thirty-nine per cent of patients had associated neurological disease; 13% had a progressive cerebellar syndrome with disabling gait ataxia, abnormal eye movements and cerebellar atrophy on neuro-imaging. BVF was usually unsuspected. Nine per cent had cranial or peripheral neuropathies and in this group there was no abnormality of brain stem/cerebellar oculomotor function, but hearing loss was common. Eleven per cent revealed BVF and hearing loss secondary to meningitis, and 6% had other neurological disorders. Idiopathic BVF was found in 21% of cases, characterised by paroxysmal vertigo and/or oscillopsia, but no abnormal clinical signs. Gentamicin ototoxicity accounted for a further 17%, while autoimmune disease was present in 9% of patients. Otological or neoplastic disease was diagnosed in the remaining 13% of patients. It was concluded that neurological, audiological and ocular motor assessments allow the probable cause of BVF to be defined in approximately 80% of cases. A group of BVF related to autoimmune pathologies is reported for the first time, indicating the need for immunological screening. Idiopathic BVF may present with only minor visual or vestibular symptoms, while in patients with cerebellar degeneration, BVF may be unsuspected and, thus, underdiagnosed.

Adult↗