Coexistence of acute hearing loss with retinal artery occlusion and encephalopathy.

We characterize 2 cases with sensorineural hearing loss and ophthalmologic findings. The clinicopathologic features revealed diagnosis of Susac's syndrome, a rare microangiopathy with cochlea, retinal, and brain affection. Diagnosis may be difficult because most specialists are not familiar with this angiopathy. However, the characteristic symptoms can mimic different pathologies, which may result in attention of radiologists, ophthalmologists, neurologists and otolaryngologists. In this report, we present 2 women with Susac's syndrome unveiled by audiometry, magnetic resonance imaging of the brain, and ophthalmologic findings. The course of the illness and a review of literature are presented.

Long-term functional outcome of macular hole surgery correlated to optical coherence tomography measurements.

OBJECTIVE: Evaluation of long-term functional outcome after macular hole surgery and its correlation with postoperative optical coherence tomography (OCT). METHODS: Before and after surgery a complete clinical examination and OCT were performed in 38 patients. Best corrected postoperative visual acuity (VA) was correlated to the following OCT parameters: preoperative base and minimum diameter of the hole, hole height, hole form factor, retinal thickness and nerve-fibre layer measurements postoperatively, postoperative foveal contour, appearance of retinal pigment epithelium layer and photoreceptor layer. RESULTS: Over a mean follow up of 67 months, VA improved significantly (P=0.01). Retinal thickness and retinal thickness and nerve-fibre layer measurements of the operated eye did not differ from fellow eye. A negative correlation was seen between final VA and preoperative base diameter of the hole (r=-0.41, P=0.02) and hole height (r=-0.45, P=0.01). The correlation between hole form factor values and final VA (r=0.36, P=0.04) was weak. Appearance of the photoreceptor band was the only OCT parameter significantly correlating with final VA (r=-0.42, P=0.01) and explaining the observed increase (r=-0.32, P=0.05) in VA. CONCLUSION: Appearance of the photoreceptor layer on postoperative OCT correlates to functional outcome and may help to explain cases of unsatisfactory postoperative VA despite successful hole closure.

Osteopontin as a serologic marker for metastatic uveal melanoma: results of a pilot study.

PURPOSE: To evaluate the protein osteopontin (OPN) as a potential new marker for screening and detection of metastatic uveal melanoma. DESIGN: Prospective, clinical study. METHODS: Twenty-eight plasma samples of 27 patients with uveal melanoma were analyzed, and the OPN plasma levels were quantified. Eight of these patients showed liver metastasis. As a control, we measured OPN levels in eight healthy, age-matched individuals. RESULTS: The median plasma concentration of OPN in patients with melanoma without metastasis was 46.78 ng/ml (range, 14.5 to 118.67 ng/ml). In contrast, increased median levels of OPN of 170.72 ng/ml (range, 87.37 to 375.54 ng/ml, P <.001) were seen in eight patients with proven metastatic disease. Healthy patients without uveal melanoma showed a median plasma concentration of OPN of 54.6 ng/ml (range, 38.23 to 71.21 ng/ml). CONCLUSION: The protein OPN seems to be a promising tumor marker for detecting metastatic disease in patients with uveal melanoma.

Visual quality of life after macular hole surgery: outcome and predictive factors.

BACKGROUND: In the present study we evaluated the functional success after macular hole surgery in correlation to visual quality of life and looked for predictive factors determining surgical success. METHODS: Fifty-nine patients that underwent pars plana vitrectomy for idiopathic macular hole were included. Follow-up visits were performed in regular intervals after surgery and included a clinical examination, optical coherence tomography (OCT) and measurement of visual acuity. To assess the visual quality of life patients filled out the National Eye Institute 25-item Visual Function Questionnaire (VFQ-25) before and three months and one year after surgery. RESULTS: Macular hole closure was achieved in 57 of 59 patients (97%). Mean visual acuity increased from 20/100 preoperatively to 20/34 one year after surgery (p = 0.02). Despite good visual acuity (20/27) in the fellow eye, visual quality of life (VFQ composite score) rose from 75.9 +/- 14.4 (SD) to 81.5 +/- 14.2 one year after surgery (p<0.001). Although there was no correlation between the increase in visual quality of life and visual acuity, the increase in VFQ-25 could be well predicted: low visual acuity and significant impairment on VFQ-25 testing preoperatively made patients most likely to benefit from macular hole surgery. A relatively high retinal thickness measurement at the hole border measured on OCT further increases the predictive value. CONCLUSION: Macular hole surgery is associated with an increase in visual quality of life despite good visual acuity of the fellow eye. Preoperative visual acuity, VFQ-25 value and partly OCT may help to predict the increase in patients' vision related quality of life after surgery.

Five-year follow-up of macular hole surgery with peeling of the internal limiting membrane: update of a prospective study.

PURPOSE: To report on long-term results of macular hole surgery with peeling of the internal limiting membrane (ILM) in a prospective nonrandomized study. METHODS: Sixty-four consecutive patients with a follow-up of at least 36 months were included. Only idiopathic macular holes were included in the study. All patients had undergone standard pars plana vitrectomy with removal of the ILM and intraocular gas tamponade with a 15% hexafluoroethane (C2F6) gas-air mixture followed by a face-down position for at least 5 days. During each follow-up visit, complete clinical examination including determination of best-corrected visual acuity, Goldmann perimetry, and optical coherence tomography was performed. RESULTS: Fifty-two patients were female and 12 were male, and the patients' mean age was 72 years (range, 53-82 years) at the last visit. We observed stage 2 holes in 5 patients, stage 3 holes in 47, and stage 4 holes in 12. The median postoperative follow-up was 62 months (mean, 56 months; range, 36-75 months). Sixty-two patients (97%) were pseudophakic at the last examination: 3 patients (5%) were already pseudophakic at the time of macular hole surgery; a combined procedure was performed on 9 patients (14%); and 50 patients (78%) underwent cataract surgery later. The median follow-up for patients after cataract extraction was 61 months (mean, 56 months; range, 36-75 months). Anatomical closure was achieved in 61 (95%) of 64 patients as confirmed clinically and by optical coherence tomography. No late reopening of a macular hole or formation of epiretinal membranes was observed after successful hole closure. Best-corrected visual acuity improved in 59 (92%) of 64 patients, remained unchanged in 2, and deteriorated in 3. Best-corrected visual acuity improved from a median of 20/100 preoperatively to a median of 20/32 postoperatively (P < 0.001). There was a median gain of 5 lines (range, -6 to 12). The development of visual acuity did not depend on the duration of symptoms, the number of surgeries, or the stage of the macular hole. CONCLUSIONS: Macular hole surgery with peeling of the ILM is a very safe procedure, even in the long term. It leads to very good and stable functional and anatomical results.

Evaluation of the internal limiting membrane after conventional peeling during macular hole surgery.

We evaluated the histologic features of the internal limiting membrane (ILM) of the retina removed during macular hole surgery without indocyanine green staining. Our investigation focused on the presence or absence of retinal structures adherent to the retinal surface of the ILM. Because only tiny retinal cellular fragments were observed especially in ILM folds, we conclude that conventional ILM peeling can be performed safely with a cleavage plane between the retinal surface of the ILM and Müller cell endfeet.