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PURPOSE: To report the histological findings and clinical course of 2 patients with microcyst-like epithelial keratopathy (MEK) associated with antibody-drug conjugate, depatuxizumab mafodotin. METHODS: Case series. RESULTS: Two patients with glioblastoma multiforme participating in a phase 3 clinical trial of the antibody-drug conjugate, depatuxizumab mafodotin, presented with bilateral MEK. Confocal imaging showed multiple large, round, hyperreflective lesions in the epithelium. Epithelial debridement was performed for symptomatic relief in both patients. Along with aggressive lubrication, bandage contact lenses, and reduction in the chemotherapeutic dose to maintenance levels, both patients experienced symptomatic improvement. However, MEK lesions recurred after re-epithelialization. Immunohistochemistry of the diseased epithelium showed immunoglobulin (Ig)G-positive granular cytoplasmic inclusions and increased cell apoptosis. CONCLUSIONS: Depatuxizumab mafodotin accumulates in the basal corneal epithelium resulting in MEK because of increased apoptosis. Frequent lubrication and bandage contact lenses can provide symptom relief.

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We describe two patients in whom postsurgical refractive error occurred after routine refractive lens exchange cataract surgery with multifocal intraocular lens insertion most likely attributable to the presence of underlying corneal epithelial basement membrane dystrophy (EBMD). In Case 1, there was an unexpected hyperopic postoperative spherical equivalent refractive error of +1.50 diopters and in Case 2, a cylindrical refractive error of 2.75 diopter cylinder. We examine the possible causes of error and discuss potential management strategies to prevent and address these unpredictable postoperative outcomes. The importance of comprehensive and careful ocular surface assessment before cataract or refractive surgery as part of the presurgical workup is reemphasized. EBMD can be subtle and if overlooked, can affect the validity of biometric keratometric measurements preoperatively, resulting in an inaccurate biometry measurement, incorrect IOL selection, and reduced visual performance and patient satisfaction.

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RATIONALE: The etiology of anterior corneal opacities and the effect of debridement cannot be determined by biomicroscopy. Optical coherence tomography (OCT) helps identify the character and depth of these lesions. PATIENT CONCERNS: A 45-year-old female complained of progressive blurred vision for a long time. Slit lamp biomicroscopy showed irregular, faint scar-like opacity of anterior cornea in her both eyes. Pentacam Scheimpflug camera tomography showed irregular astigmatism of anterior corneal surface. Anterior segment spectral-domain OCT revealed thickened, hyper-reflective linings, and scattered lesions, mainly in the epithelial layer. DIAGNOSES: Epithelial basement membrane dystrophy (EBMD). INTERVENTION: Epithelial debridement and bandage lenses. OUTCOMES: The cornea became clear and the vision improved soon after debridement. The pathology showed thickened aberrant basement membrane extending into mid-epithelial layer, with microcyst-like lesions also noted. LESSONS: OCT defines the depth of lesions and helps diagnosis and management of EBMD.

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The objective of this study was to evaluate the long-term outcomes of patients with Cogan syndrome (CS) who have undergone cochlear implantation. Subjects consisted of 12 cochlear implant users with a typical form of CS. Measures included word and sentence recognition scores. The speech recognition performance was rated before cochlear implantation and at 1 and 5 years after implantation. The speech materials were presented in quiet only condition. The mean 12-month post-operative word and sentence recognition scores were 91.4 and 93.1%, respectively. Five years after implantation, the group means for word and sentence recognition tests were 94 and 96.3%, respectively. No patients in this series experienced flap complication or other local or systemic complications. This long-term study on 12 subjects with CS over 5 years of cochlear implant use reveals that cochlear implantation is safe in the long term and provides excellent and stable hearing results.

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OBJECTIVES/HYPOTHESIS: Evaluate the characteristics and outcomes of patients with autoimmune inner ear disease (AIED) who have undergone cochlear implantation (CI) and compare post-CI performance in AIED to matched controls. STUDY DESIGN: Retrospective case control study. METHODS: Study cohort was comprised of 25 adult implantees (AIED [n = 18], Cogan syndrome [n = 7]). The AIED group was defined by rapidly progressive bilateral sensorineural hearing loss leading to unusable hearing within weeks to months. Patients with Cogan syndrome, the archetypal inner ear autoimmune disease, were also examined and used for within-cohort comparison. Clinical and operative records were reviewed. Post-CI performance was assessed using open-set sentence tests. Age- and sex-matched individuals deafened by other postlingual causes were used as controls. RESULTS: Of 25 patients, 24 had uneventful, full electrode insertions. One AIED patient had partial insertion due to cochlear ossification and did not achieve open-set speech perception post-CI. Mean open-set sentence scores for study patients with uneventful insertions were 92.8%, 97.3%, and 96.4% at 6 months, 1 year, and ≥ 2 years, respectively. Compared to matched controls, patients deafened by autoimmune causes had significantly higher post-CI performance at all postoperative test intervals (P < .05). There was no significant difference in postimplantation performance between Cogan syndrome and AIED patients. CONCLUSIONS: To our knowledge this was the largest study of cochlear implantation in AIED and Cogan syndrome patients. In our experience, both groups generally attained high levels of post-CI speech perception and performed above average. Cochlear ossification affecting implantation in Cogan syndrome patients was not observed in our series, contrary to some reports.

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PURPOSE: The evaluation of long-term results of cochlear implantation in patients with typical Cogan syndrome. PROCEDURES: The medical records of approximately 3,000 patients who underwent cochlear implantation in a tertiary centre within the period 1992-2007 were retrospectively examined. RESULTS: Four cochlear implant recipients with Cogan syndrome (6 implantations) were identified. One cochlea was found to be partially obliterated; the electrode could, however, be completely inserted. Wound healing disorders and infections in a patient with systemic symptoms were the only postoperative complications. Hearing outcome was favorable, with average HSM and monosyllabic scores of 96.7 and 82.5%, respectively, in an average follow-up time of 9.25 years. CONCLUSIONS: Cochlear implantation is the appropriate hearing rehabilitation method in Cogan syndrome patients. Although the basic illness does not affect the long-term hearing outcome, skin-atrophy-related complications in cases with systemic symptoms may occur. Finally, the cochlear implant surgeon should be aware of the possible cochlea obliteration.

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BACKGROUND: Cogan's disease is a diagnostic challenge. The typical form of this chronic inflammatory disease is characterized by non-syphilitic interstitial keratitis and acute audiovestibular impairment. CASE REPORTS: The first patient had "typical" symptoms of Cogan's syndrome with recurrent keratoconjunctivitis, severe hearing loss and vertigo. Systemic cardiovascular manifestations occurred in the further course of the disease with multiple vascular stenoses of the carotids and vessels of the lower limbs. The second patient had "atypical" symptoms of Cogan's disease with progressive bilateral hearing loss and vestibular deficits, unilateral conjunctivitis and orbital swelling and unspecific symptoms, such as fever and headaches. Therapy with systemic and local corticoids was successful in both cases concerning ocular and systemic symptoms. The first patient had received additional therapy with methotrexate, but had already become deaf before therapy had started. Cochlear implantation was successfully carried out. In the second patient hearing loss was stationary under therapy and the patient could be provided with hearing aids. CONCLUSION: Because of obligatory cochleovestibular symptoms the otorhinolaryngologist has a key position in diagnosis and therapy of this rare entity. Early diagnosis is a crucial prognostic factor concerning preservation or restitution of hearing function.

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