The pattern ERG in Best's disease

The aim of this study was to assess inner retinal function in patients with Best's disease using the pattern ERG (PERG). Nine patients with Best's disease, who had good visual acuity, were studied. Five of the nine had abnormal PERGs. All five had some reduction in central visual acuity. We believe that the abnormal PERGs in these patients represents photoreceptor cell loss which is occurring at an early stage in Best's disease.     

A comparison of pattern and multifocal electroretinography in the evaluation of age-related macular degeneration and its treatment with photodynamic therapy

This study compares pattern electroretinography (PERG) and multifocal electroretinography (mfERG) measures in 13 patients with predominantly classic choroidal neovascularisation (CNV) associated with age-related macular degeneration (ARMD, 9/13 unilateral, 4/13 bilateral), assesses the usefulness of each test in monitoring disease progression, and identifies electrophysiological predictors of outcome following treatment with photodynamic therapy (PDT). PERG and mfERGs were recorded at presentation, 2 weeks post-treatment, and at 3 monthly intervals for 2 years. The PERG was detectable in 8/13 patients with unilateral disease; the mfERG was detectable in 12/13 patients. P50 and N95 amplitudes increased in 6/8 patients and mfERG p1 increased in 7/13 patients at 2 years. PERG amplitudes correlated strongly with mfERG amplitudes in patients with unilateral disease. PERG P50 and mfERG p1 amplitude correlated with visual acuity at 2 years (R = 0.68, R = 0.82, respectively). The largest PERG P50 and mfERG p1 amplitude difference between treated and fellow eyes of all the groups on initial visit was associated with a poor visual outcome (P50 64% difference; p1 29% difference) whereas those with the smallest P50 and p1 amplitude difference was associated with improved vision at 2 years (P50 30% difference; p1 21% difference). The PERG and mfERG provide an objective measure of central retinal function in the progression of ARMD. A detectable PERG on presentation was the single best indicator of improved function and visual acuity at 2 years. The mfERG demonstrated disease progression from central retina into the paramacular regions over 2 years. Patients with poor visual outcomes had the largest inter-ocular amplitude difference on presentation, suggesting that such patients may have a worse prognosis following treatment.In part presented at ARVO 2004 (Fort Lauderdale, USA) and ISCEV 2004 (Peurto Rico, USA).     

Local macular ERG in patients with Best's disease

We examined a pair of siblings, a 10-year-old girl (case 1) and a 12-year-old girl (case 2), with Best's diease. The visual acuity was nearly normal in both patients. The central visual field measured with auto-plot tangent screen revealed a small relative paracentral scotoma only in the left eye of case 2. We failed to detect any abnormality in photopic and scotopic electroretinogram (ERG) recorded with Ganzfeld stimuli and the electrooculogram light rise was absent in both patients.Local macular ERG was recorded under a fundus monitor by infrared television fundus camera with test spots of 5, 10, and 15 degrees in diameter. The center of the stimulus spot was always on the fovea during the recording. The local macular ERG was absent in both patients. Our results of local macular ERG may indicate disturbance of the central portion of the retina.     

A 43-year-old man with reduced visual acuity and normal fundus: occult macular dystrophy—case report

Purpose Occult macular dystrophy (OMD) is an unusual, inherited macular dystrophy characterized by a slowly progressive decline of visual acuity with normal fundus and fluorescein angiography (FA). The authors present a 43-year-old man who was diagnosed as having OMD because of the results of electrophysiological, psychophysical, optical coherence tomography (OCT) tests. Methods Routine ophthalmological evaluation, FA, visual field tests, electroretinographic examinations (EOG, ERG, PERG and mfERG recordings according to ISCEV standards) and foveal thickness measurements (OCT) were performed. Results Funduscopic examinations, FA, full field ERG as well as PERG results were all normal. In both eyes, the abnormalities were observed in static perimetry (relative central scotomas), mfERG (significant reduction of P1 amplitude in the central retinas) and OCT (significantly thinner foveal thickness). Conclusions A new case with OMD is added to preceding reports. The mfERG and OCT tests are important in detection of OMD patients. It can help in differential diagnosis of amblyopia, optic nerve diseases and non-organic visual disorders.     

服用羟氯喹者的视觉电生理检查

目的 对服用羟氯喹者进行视网膜电图和眼电图检查,评价用药安全性.方法 选择正在服用羟氯喹的180例自身免疫病患者,行视力、眼压、眼前节及散瞳眼底检查,其中55例患者行视网膜电图和眼电图检查.每6个月复诊1次.结果 180例患者的视力、眼压、眼底均正常,55例患者按用药时间分组,各用药时段组Arden比、a波、b波峰时和a波平均振幅及30 Hz反应差异均无统计学意义(P>0.05),而在用药13～24月组,-15dB光刺激强度下的b波平均振幅较未用药组和用药3～12月组差异均有统计学意义(P<0.05).结论 使用低剂最的羟氯喹具有良好的眼部安全性.     

The pattern electroretinogram in patients with optic nerve disease

We analyzed the electroretinogram elicited by pattern stimuli (PERG) in 29 patients with unilateral optic nerve disease. The normal fellow eyes served as a control. When the normal fellow eye was covered and the patient was asked to watch the center of the pattern stimuli with the affected eye, the poor fixation often caused irregular eye movements which resulted in a poor reproducibility of the response. In such instance we opened the normal fellow eye for fixation, which helped to stabilize the affected eye being examined even when visual acuity was extremely poor. The cross-talk phenomenon of the ERG and the reflection of the VER from the normal fellow eye were proved negligible in this recording condition.We analyzed PERGs in comparison with the visual acuity and the degree of optic atrophy. Unlike the results of many previous authors, little correlation was observed between PERG and these two factors. Most patient showed normal or only slightly abnormal PERG, even when the visual acuity was extremely poor and the optic disc was completely atrophic.Our results suggest that, at least with our method, PERG has extremely limited value in detection of optic nerve disease.     

Fundus autofluorescence imaging in Best's vitelliform dystrophy

BACKGROUND: It is well known that with age lipofuscin accumulates in the retinal pigment epithelium (RPE). In many inherited macular dystrophies such as Best's disease abnormally high levels of lipofuscin were found by histopathological studies. In recent years, it has become possible to detect and image fundus autofluorescence in the living eye as an index of lipofuscin accumulation in the RPE. The aim of our study was to document the intrinsic fundus autofluorescence in patients with different stages of Best's disease and to correlate fundoscopic features with visual function in these patients. METHODS: Images of fundus autofluorescence were obtained from 10 patients with Best's vitelliform dystrophy, using a Heidelberg Retina Angiograph (HRA). Argon laser light (488 nm) was used for illumination, and a wide-bandpass filter with a cutoff of 500 nm was inserted in front of the detector to obtain the autofluorescence images. Images were compared with fundus appearance and fluorescein angiograms as well as with visual acuity, colour vision, visual fields and electrophysiological recordings in these patients. RESULTS: In initial stages of Best's disease, there were localised areas of hyperfluorescence centrally in the fovea, later being replaced by central hypofluorescence (atrophic) areas surrounded by hyperfluorescent rings. The evolution of autofluorescence pattern showed centrifugal spreading of retinal dysfunction from the centre towards periphery. Atrophic regions of the RPE were associated with low levels of background autofluorescence, lower visual acuity, abnormal colour vision (70% of patients), central scotomas (85% of patients) and poorer electrophysiological results (reduced PERG responses in 45% of patients with Best's disease). CONCLUSIONS: Fundus autofluorescence imaging provides new information regarding the content and spatial distribution of RPE lipofuscin in eyes with Best's disease, which appears to correspond to retinal function. This may "in vivo" give important clues to the pathogenesis and progression of Best's disease in which non-invasive autofluorescence imaging may replace fluorescein angiography.     

Pattern electroretinogram (PERG) in the early diagnosis of normal-tension preperimetric glaucoma: a case report

Introduction

The purpose of this case is to present the use of pattern electroretinogram (PERG) in the early diagnosis of normal-tension preperimetric glaucoma in 56 years old woman.

Methods and Results

At baseline the results were as follows: distance-corrected visual acuity in the right eye (RE) and left eye (LE) 1.0 and 0.7, respectively (Snellen table), normal anterior segments in both eyes, normal fundus in the RE and abnormal cup to disc ratio (0.6) in the LE. Intraocular pressure (IOP) was within normal limits in both eyes: RE-14 mmHg, LE-18 mmHg (Goldmann tonometer). Results of standard automated perimetry (SAP), short wavelength automated perimetry (SWAP) and nerve fiber analyzer (GDx) were normal in both eyes. PERG result was normal in the RE but in the LE reduced amplitudes of P50 and N95 waves were observed. After topical treatment (Xalacom to the LE), a reduction of IOP to 13 mmHg was achieved and was accompanied by amplitudes increase of PERG waves. After discontinuation of the therapy, IOP increased to 18 mmHg and P50 and N95 amplitudes decreased to the values before treatment, suggesting the influence of IOP lowering therapy on electrical function of retinal ganglion cells. After 4 years from the baseline, static perimetry results were still normal, but abnormalities in retinal nerve fiber layer thickness were detected in GDx.

Conclusions

PERG was a useful test not only for the early diagnosis of normal-tension preperimetric glaucoma, but also in evaluating the effectiveness of antiglaucomatous treatment.     

图形视网膜电图检测原发性开角型青光眼视网膜功能的意义

目的 观察原发性开角型青光眼图形视网膜电图的改变及其特点,了解ＰＥＲＧ在检测ＰＯＡＧＰＹ视网膜功能方面的意义。方法 对３６例（５９只眼）ＰＯＡＧ及３２例（５９只眼）年龄相匹配的正常人进行ＰＥＢＧ检测。结果 ＰＯＡＧ患者ＰＥＲＧ的ＡＰ１、ＡＮ２、ＡＮ１、ＡＮ２／ＡＰ１下降;运用ＡＮ２＋ＡＰ１〈２．７及ＡＮ２／ＡＰ１下降;运用ＡＮ２＋ＡＰ１〈２．７及ＡＮ２／ＡＰ１〈０．７的方法,对已确认的ＰＯＡＧ进行     

Symptomatic and asymptomatic visual loss in patients taking vigabatrin.

PURPOSE: To investigate the clinical, perimetric, and electrophysiologic findings in patients with visual field loss on long-term treatment with the antiepileptic medication vigabatrin. DESIGN: Consecutive observational case series. PARTICIPANTS: Forty-one consecutive subjects taking vigabatrin referred for screening ophthalmologic assessment were studied. Twelve subjects with evidence of peripheral visual field constriction are presented. METHODS: Twelve subjects with evidence of peripheral visual field constriction on 60-4 perimetry underwent central 30-2 and blue-on-yellow (B/Y) perimetry, as well as electroretinography (ERG), electro-oculography (EOG), and visual-evoked potential (VEP) testing. MAIN OUTCOME MEASURES: Visual acuity; fundus abnormalities; visual field loss; and ERG, EOG, or VEP abnormalities were the main outcome measures. RESULTS: Eight of the 12 subjects with constricted visual fields were asymptomatic. The central 30-2 perimetry demonstrated bilateral visual field constriction in 9 of 12 patients and the B/Y perimetry in 8 of 9 patients tested. Of the ten patients tested electrophysiologically, four had abnormal ERGs, five had abnormal EOGs, and three had delayed VEPs. CONCLUSIONS: The incidence of visual field constriction in patients taking vigabatrin may be higher, and asymptomatic visual field loss more common, than reported previously. The authors postulate a possible Muller cell dysfunction in the peripheral retina. Patients taking vigabatrin should have regular peripheral visual field examinations.     

The pattern electroretinogram in glaucoma patients with confirmed visual field deficits

PURPOSE: To better understand the relationship between the amplitude of the pattern electroretinogram (PERG) and visual loss, measured with static automated perimetry. METHODS: Transient PERGs were recorded in 15 patients (31-77 years) and 16 normal individuals (26-65 years). An eye was considered to have glaucomatous damage only if there was an abnormal disc, an abnormal 24-2 Humphrey visual field result (pattern stand deviation, glaucoma hemifield test, and cluster) and an abnormal multifocal visual evoked potential. All the worse (more affected) eyes of the patients and six of the better eyes met these criteria. The N95 amplitude of the PERG was measured from the positive peak (P50) at approximately 50 ms to the trough at approximately 95 ms. The ratio of N95 to P50-the N95 amplitude divided by the P50 amplitude-was also measured. RESULTS: First, the PERG was within normal limits for 4 (26.7%) of the worse eyes. Overall, 6 (28.6%) of the 21 eyes that met the criteria for glaucomatous damage had normal PERGs on both PERG measures. Because the normal individuals were younger than the patients, an even larger number of normal PERGs might be expected with an age-appropriate control group. Second, the N95 amplitude was nonlinearly related to visual field sensitivity when sensitivity was plotted on a linear plot. Small field losses were associated with disproportionately large losses in PERG amplitude. Third, the PERG from both eyes of a patient were very similar, even when the visual fields suggested very different levels of damage. CONCLUSIONS: These results are consistent with the view that very early damage can affect the PERG, even before the visual field shows a loss. At the same time, it is clear that patients with clear glaucomatous damage can have normal-appearing PERGs. An explanation is proposed to account for these findings.     

Diagnosis of retinal diseases. Comparison between multifocal ERG and fundus perimetry - a case study

Background. There are new methods available for function testing of the macula, i.e. multifocal Electroretinography (mfERG) as well as fundus perimetry with the scanning laser ophthalmoscope (SLO). The value and clinical impact of these methods have still to be evaluated. We wanted to compare the results from patients having undergone both examinations. Patients and methods. A total of 33 eyes from 25 patients (visual acuity 0.03–0.8) aged 14–79 years were examined using fundus perimetry with the SLO. In addition, multifocal ERG was performed in all eyes, where 61 local ERGs inside the 30° visual field were recorded. We compared the depth of the scotoma with the reduction of the amplitudes during mfERG taking the fixation status into consideration. Results. Examination time was comparable for both examination techniques. There was good concordance for eyes with retinitis pigmentosa with only central response. In contrast, patients with juvenile macular dystrophy demonstrated different results with comparable size of the defect while sometimes an enlarged pathology during mfERG was observed. Correlation between pathology findings observed in mfERG and fundus changes was difficult due to the change of the point of fixation caused by central scotoma. In age-related macular degeneration correlation of the findings was less obvious. Discussion. The different setting with supra-threshold stimuli during ERG in contrast to near-threshold stimulus presentation during perimetry, might be the reason for differences even in the beginning of retinal diseases. In addition, reduced stability of fixation leads to artifacts during mfERG while it may be compensated for in fundus perimetry. Both methods are of additional value and demonstrate different results depending on the disease.     

Pattern electroretinograms for the detection of neural loss in patients with permanent temporal visual field defect from chiasmal compression

Purpose To evaluate the ability of full-field and hemifield pattern electroretinogram (PERG) parameters to differentiate between healthy eyes and eyes with band atrophy (BA) of the optic nerve. Methods Twenty-six eyes from 26 consecutive patients with permanent temporal hemianopic visual field defects and BA of the optic nerve from previous chiasmal compression and 26 healthy subjects were studied prospectively. All patients were submitted to an ophthalmic examination including Humphrey 24-2 SITA Standard automated perimetry. Full-field and hemifield (nasal and temporal) stimulation transient pattern electroretinograms (PERG) were recorded using checkerboard screens. Amplitudes and peak times for the P50 and N95 as well as the overall P50+N95 amplitude were measured. The intraocular N95:P50 amplitude ratio was calculated. Comparisons were made using Student’s t-test. Receiver operating characteristic (ROC) curves were used to describe the ability of PERG parameters to discriminate the groups. Results Full-field P50, N95, and P50+N95 amplitude values were significantly smaller in eyes with BA than in control eyes (P < 0.001). Nasal and temporal hemifield PERG studies revealed significant differences in N95 and P50+N95 amplitudes measurements. No significant difference was observed regarding peak times or N95:P50 amplitude ratios. Nasal and temporal hemifield PERG values did not differ significantly in eyes with BA or in controls. Using the 10th percentile of normals as the lower limit of normal, 16 of 26 eyes were considered abnormal according to the best discriminating parameters. Conclusions Transient PERG amplitude measurements were efficient at differentiating eyes with BA and permanent visual field defects from normal controls. Hemifield stimulation PERG parameters were unable to detect asymmetric hemifield neural loss, but further studies are required to clarify this issue. Study registered on ClinicalTrial.gov number: NCT00553761     

The pattern electroretinogram in optic nerve disease

Pattern evoked electroretinograms (PERG), diffuse flash electroretinograms (ERG) and visual evoked potentials were studied in patients with unilateral optic nerve disease. Patients with Snellen acuities of less than 6/30 did not have recordable PERGs in their affected eye, whereas their diffuse flash ERGs were normal. The VEPs were correspondingly reduced or absent when recorded from the poorer seeing eyes. A second group of patients with Snellen acuity between 6/6 and 6/30 in the involved eye showed reductions in the mean PERG amplitude of the affected as compared with the normal eyes. All affected eyes showed an abnormal contrast threshold measured with the PERG amplitude. Such results underscore the diagnostic value of the PERG in detecting even mildly affected cases of optic nerve disease.     

Abnormal fundus autofluorescence in relation to retinal function in patients with retinitis pigmentosa

Background Fundus autofluorescence (AF) in some patients with retinitis pigmentosa is characterized by a parafoveal ring of increased AF which surrounds the centre as hypofluorescent changes appear at the periphery. The aim of this study was to evaluate the AF patterns in relation to retinal function measured by electroretinography and visual fields. Methods Thirty-two patients with RP were included in the study. AF imaging of the macular area was performed with the scanning laser ophthalmoscope. Patients were divided in two groups according to their fundus AF patterns. All patients from group 1 had a ring of increased AF of different size but no atrophic areas inside vascular arcades. Patients with a ring of increased AF and round atrophic changes at different eccentricities from their fovea were selected in group 2. Visual fields were tested with kinetic, automated perimetry and microperimetry; the radius of the hyperfluorescent ring and the smallest distance of hypofluorescent areas from the fovea were compared to visual fields, PERG P50 and N95 and mfERG P1 amplitudes of the inner three rings. Results A linear relationship was found in group 1 between the radius of the ring of increased AF and both the automated (r=0.82) and kinetic perimetry (r=0.80). The radius of the AF ring correlated highly with the PERG P50 (r=0.72) and N95 (r=0.74) amplitudes. In all patients, mfERG responses were reduced at all retinal locations, more pronounced at periphery. There was a good correlation between the ring of increased AF and the P1 amplitude of ring 2 of mfERG (r=0.62). Patients from group 2 had significantly reduced or non-recordable PERGs and mfERGs. The eccentricities of hypofluorescent changes did not correlate with any type of perimetry. Conclusions Our results show that in stages of retinitis pigmentosa, before atrophic lesions spread inside the vascular arcades, the pattern of fundus autofluorescence correlates well with functional tests such as perimetry and electroretinography. The ring of increased AF appears to represent the border between functional and dysfunctional retina. This shows that autofluorescence, as a quick and non-invasive imaging tool, may be related to retinal function as well.     

Cancer-associated retinopathy (CAR) with electronegative ERG: a case report

Cancer-associated retinopathy (CAR) should be suspected in patients who present with visual symptoms such as rapid unexplained visual loss and seeing shimmering lights, with an abnormal ERG. Electronegative ERG responses are not exclusive to melanoma-associated retinopathy (MAR) but may be seen in CAR as well. We describe a patient with CAR who presented with an electronegative ERG. A 67-year old woman, who presented with complaints of seeing shimmering lights, underwent an extensive ophthalmological and electrophysiological examination. Best-corrected visual acuity was 7/10 in the right and 9/10 in the left eye. Goldmann visual fields showed relative central scotomata and concentric narrowing. Slit-lamp and fundus examination were normal as was fluorescein angiography. Rod-specific ERG responses were severely reduced, with electronegative maximal combined rod-cone responses and delayed cone-responses with normal amplitudes. Melanoma-associated retinopathy was suspected. Extensive dermatological and internal evaluation eventually revealed an oat-cell carcinoma in the right lung. The patient died of pneumonia 2 years after presentation.     

The results of electrophysiological tests in unilateral extensive peripapillary myelinated nerve fibers: report of three cases

The purpose of this study is to describe clinical and electrophysiological findings of three patients with unilateral extensive peripapillary myelinated nerve fibers. Three patients with unilateral extensive peripapillary myelinated nerve fibers were examined. A complete ophthalmologic examination including uncorrected and best spectacle corrected visual acuities (UCVA, BSCVA) on Snellen charts, dilated fundoscopy, fundus fluorescein angiography (FFA), optical coherence tomography (OCT), perimetry and electrophysiological tests was performed. ERG results of both eyes were normal in all cases. FFA showed hypofluorescence at the myelinated nerve fibers (MNF) area in the affected eyes. We found a visual field defect resembling arcuat scotom at perimetry of the affected eyes. In OCT, there was hyper-reflectivity in reply to MNF. In all cases, pattern ERG results of the affected eyes showed decreased amplitudes and increased latencies of P50 and N95 waves. P100 amplitudes of PVER and FVER were decreased, and latencies were increased in all affected eyes. Extensive peripapillary myelinated nerve fibers may cause changes in PERG and VER.     

Short wavelength automated perimetry, frequency doubling technology perimetry, and pattern electroretinography for prediction of progressive glaucomatous standard visual field defects

PURPOSE: To evaluate the clinical use of a test battery of short wavelength automated perimetry (SWAP), frequency doubling technology perimetry (FDT), and pattern electroretinography (PERG) in predicting progressive glaucomatous visual field defects on standard automated perimetry (SAP). STUDY DESIGN: A prospective, longitudinal, observational case series. PARTICIPANTS: One hundred and fifty-two patients with primary open-angle glaucoma (POAG) with bilateral glaucomatous visual field defects on SAP were followed at 6-month intervals over a period of 30 months. MAIN OUTCOME MEASURES: Short wavelength automated perimetry, FDT, and PERG results were compared between POAG eyes with and without progressive field loss on SAP. These two groups were used to evaluate whether PERG, SWAP, and/or FDT is predictive of future progression of field loss on SAP. RESULTS: Using the criteria of progressive field loss on SAP defined by the Collaborative Normal Tension Glaucoma Study, 54 eyes (study group) of 54 POAG patients showed progressive defects, whereas 84 eyes (control group) of 84 POAG patients showed no progression. Only 11.1% (6 of 54) of the eyes with a progression of field loss on SAP showed no increase of deficits on the three functional tests before progression. Short wavelength automated perimetry detected early progressive defects on SAP in 43 of the 54 eyes (79.6%). Of these 54 POAG eyes, FDT showed progressive deficits in 40 eyes (74.1%), whereas PERG amplitude P1N2 showed progressive deficits in 35 eyes (64.8%) before progression of field loss on SAP. A test battery consisting of SWAP and PERG P1N2-amplitude was able to detect 88.9% of eyes before a prediction of field loss on SAP. When comparing the results of the two functional tests, SWAP and FDT in the 84 eyes without progression of field loss on SAP between baseline and at 30 months, SWAP and FDT showed progressive deficits in 34.5% and 35.7%, respectively. CONCLUSIONS: All three tests (SWAP, FDT, and PERG) have been successful in detecting glaucoma eyes with a future progression of standard visual field defects. A test battery of SWAP and PERG P1N2-amplitude improved the power to predict these progressive defects on SAP. It remains to be seen whether the long-term follow-up in POAG eyes will improve the false-positive rate of SWAP and FDT.     

Visual prognostic value of the pattern electroretinogram in chiasmal compression

BACKGROUND/AIMS: The visual loss associated with compression of the optic chiasm by pituitary tumours may be transient or permanent, possibly related to the extent of irreversible retrograde degeneration to the retinal ganglion cells. The pattern electroretinogram (PERG) N95 component is thought to rise in relation to retinal ganglion cell function and hence may be a potential prognostic indicator for visual function following decompressive surgery. METHODS: The notes and electrodiagnostic records of 72 eyes from 36 patients with chiasmal compression were retrospectively analysed. RESULTS: The postoperative change in visual field was found to be associated with the PERG N95:P50 ratio (p=0.01). Improvement in visual field was shown by a greater proportion of eyes with a normal N95:P50 ratio (65%) than with an abnormal ratio (27%). No change in visual field occurred in 26% of the eyes with a normal N95:P50 ratio compared with 67% of those with an abnormal ratio. Only 8% of eyes showed a worsening of visual field following surgery, in similar proportions for eyes with normal and abnormal N95:P50 ratios. There was no significant relationship with visual acuity. CONCLUSION: The PERG is a useful visual prognostic indicator in the preoperative assessment of chiasmal compression.     

Assessing Responses of the Macula in Patients with Macular Holes using a New System Measuring Localized Visual Acuity and the mfERG

Purpose: To evaluate acuity and multifocal electroretinogram (mfERG) responses from the macula in affected and unaffected fellow eyes of patients with macular holes. Methods: We tested 10 eyes with macular hole and 10 fellow eyes from 11 patients. We measured local visual acuity thresholds at 27 discrete locations within 21° diameter using the Functional Fundus Imaging System (FFIS), a psychophysical system that measures visual acuity as a function of visual field location, and local ERG responses within 45° diameter using the mfERG. Results: In the affected eyes, the mean FFIS visual acuity thresholds were significantly elevated within the central 21° diameter area, compared to a group of control eyes. No significant differences were found between the acuities of the fellow eyes compared to those of the control group. The amplitudes of the first positive peak of the mfERG were reduced in the central 7.8° in affected eyes. In the central 2°, 4 out of 10 affected eyes showed non-measurable ERG signals. The remaining six eyes showed significantly reduced mean amplitudes, but not delayed implicit times, when compared to the control group. For the fellow eyes, the mean amplitudes of the mfERG and implicit times did not differ from the means of the control eyes. Conclusions: Both local psychophysical and electrophysiological testing demonstrated retinal dysfunction extending beyond the site of the macular holes in some patients (three of the patients had central mfERG amplitudes falling within the normal range).