Search

Degenerative or hereditary corneal diseases are sometimes difficult to discriminate. Corneal dystrophies affect approximately 0.09 % of the population. They are identified by the IC3D classification based on their phenotype, genotype and evidence gathered for their diagnosis. Practically, the ophthalmologist manages functional symptoms, such as recurrent erosions, visual loss and amblyopia, photophobia, foreign body sensation, and sometimes pain and aesthetic concerns. Medical treatments consist of drops to promote healing, ointments, hyperosmotic agents and bandage contact lenses. Less invasive surgical treatments are used as second line therapy (phototherapeutic keratectomy, lamellar keratectomy). More invasive procedures may eventually be utilized (lamellar or penetrating keratoplasty). Anterior lamellar or endothelial keratoplasty are now preferred to penetrating keratoplasty, although the latter still remains the only possible option in some cases. Some rare dystrophies require coordinated and comprehensive medical care., (Copyright © 2017 Elsevier Masson SAS. All rights reserved.)

A 48-year-old woman with no significant past history underwent bilateral simultaneous laser in situ keratomileusis for correction of her myopia. On the tenth postoperative day, the patient complained of visual decrease and photophobia. Slit lamp exam showed corneal epithelial irregularities. Confocal microscopy was performed and revealed a characteristic appearance of epithelial basement membrane dystrophy (EBMD). The patient was successfully treated with artificial tears and autologous serum eyedrops. EBMD may be missed before LASIK surgery, even after a careful pre-operative examination. Exacerbation of EBMD after LASIK surgery is rare. It should be considered when unexplained corneal epithelial defects or irregularities occur following LASIK. Confocal microscopy is very useful to confirm the diagnosis., (Copyright © 2013 Elsevier Masson SAS. All rights reserved.)

Cogan's syndrome is a rare disorder of unknown origin characterized by inflammatory ocular disease (mainly interstitial keratitis) and audiovestibular symptoms (mainly acute-onset sensorineural hearing loss) in the setting of a negative work-up for syphilis. The diversity of the ocular and audiovestibular manifestations reported in the literature in atypical Cogan's syndrome should make one cautious before accepting the diagnosis as the disease may mimic various other systemic disorders. Systemic corticosteroids are always the most widely used and successful therapy. For patients requiring high and prolonged doses, additional immunosuppression is appropriate. Methotrexate is the first-line steroid sparing agent. However, patients without systemic disease or severe eye disease unmanageable by topical corticosteroids should not be subjected to protracted courses of corticosteroids or immunosuppressive agents, particularly when little gain in hearing is obtained with their use. The effect of TNF-alpha blockers was recently investigated. Infliximab might be an alternative therapy in cases of failure of corticosteroids and immunosuppressive therapy. However, treatment might be more effective when started at an early stage of the disease, when the lesions are still reversible., (Copyright © 2013 Elsevier Masson SAS. All rights reserved.)

The association of visual and auditory impairments, simultaneously or consecutively, is a rare condition at the onset of neurological diseases. To determine whether audiovisual impairment can be associated with a specific group of neurological disorders at onset, we performed a prospective study of 307 patients over 6 months in a specialized neurological unit in inflammatory diseases. Six patients (2%) experienced inaugural audiovisual impairments. The mean age of patients at onset was 39.5 ± 14.7 years, with a male:female ratio of 1:2. Both deficiencies were reported in three cases, including loss of visual acuity with tinnitus (two cases) or hearing loss (one case). Initial visual dysfunction, characterised by loss of visual acuity, was noted in one patient. Initial auditory impairment, characterised by dizziness and hearing loss, was noted in two patients. The mean interval between the occurrence of visual and auditory impairments was 3.8 ± 4.3 months. A neurological diagnosis was made in four cases (67%) at a mean time of 4.6 ± 4.6 months after disease onset. Visual impairments were optic neuritis for multiple sclerosis, serous retinal detachment for Vogt-Koyanagi-Harada's disease, a central retinal artery occlusion for Susac's syndrome and a retinal vasculitis for Cogan's syndrome. The systematic investigation of inaugural audiovisual impairment in young patients could help shorten the time to a specific neurological diagnosis., (Copyright © 2010 Elsevier Masson SAS. All rights reserved.)