Jelka G. Orsoni

Cochlear Implantation and Cogan Syndrome

Objective To evaluate outcomes and issues pertaining to cochlear implantation in a group of subjects affected by Cogan syndrome. Study Design Prospective cohort. Setting Department of Ophthalmology and Otorhinolaryngology, University of Parma. Patients Five postlingually deafened adults suffering from a typical form of Cogan syndrome who underwent cochlear implantation. Main Outcome Measures Benefit from cochlear implantation as measured by word and everyday sentence recognition tests. Surgical issues and postoperative complications were also evaluated. Results In two cases, intracochlear electrodes were inserted into the scala vestibuli because of the ossification of the scala tympani. Two patients experienced a recurrence of keratitis the day after surgery. To date, with a follow-up of 1 to 4 years, no patient has experienced flap complications or other local or systemic complications. At the 12-month postoperative evaluation, all patients had gained useful open-set speech perception, achieving a mean score of 91% and 95% on word and everyday sentence recognition tests, respectively. Conclusions Patients deafened by Cogan syndrome demonstrated high levels of speech understanding after undergoing cochlear implantation. Obliteration of the cochlea may complicate electrode implantation, requiring modifications of the surgical technique. Stress consequent to the surgical procedure may instigate an acute phase of the basic illness.

Rituximab ameliorated severe hearing loss in Cogan's syndrome: a case report

BackgroundRituximab is a monoclonal antibody inducing depletion of B lymphocytes and presently approved for the treatment of non-Hodgkins lymphoma and rheumatoid arthritis. Here is the first report of the use of this drug in a case of Cogans syndrome (CS).Case Presentationa 25-year-old Italian woman was referred with conjunctival hyperaemia, interstitial keratitis, moderate bilateral sensorineural hearing loss accompanied by tinnitus, dizziness, nausea and vertigo, poorly responsive to oral and topical steroidal therapy. Diagnosis of typical CS was made. The administration of a combined immunosuppressive treatment resolved ocular inflammation, dizziness, nausea, and vertigo but gave little results in controlling progressive hearing loss. A noticeable improvement in hearing function was documented by pure tone audiometry after infusion of Rituximab.Discussionin CS, hearing function is often the most difficult parameter to control with therapy. A positive effect of Rituximab on was observed in our case. The drug also allowed to significantly reduce the number of adjuvant immunosuppressive medications.

Prospective, Multicenter Demographic and Epidemiological Study on Vernal Keratoconjunctivitis: A Glimpse of Ocular Surface in Italian Population

Purpose: To evaluate the frequency and epidemiological features of vernal keratoconjunctivitis (VKC) in Italy. Methods: a specific electronic clinical chart for vernal keratoconjunctivitis was created to standardize: 1) medical history; 2) diagnostic criteria; 3) signs and symptoms; and 4) treatments. This study involved 6 Italian referral centers for ocular surface diseases: between March 2005 and March 2006, all referred patients were included, clinical data collected and statistically examined. Results: The mean age of the vernal keratoconjunctivitis population (n = 156) was 13.8 ± 8.8 with 64.1% of subjects under 14 years of age and a male/female ratio of 3.5:1. Among VKC patients, 48.7% showed associated systemic allergic diseases. Only 32.1% of patients were positive for RAST and/or prick test. The limbal form (53.8%) was the most frequent subtype of vernal keratoconjunctivitis. Approximately 9% of patients showed a severe form of vernal keratoconjunctivitis. At the first visit patients were treated with: multiple action or mast cell stabilizer eye drops (58.1% and 41.3% of cases, respectively), topical corticosteroids alone (0.6%) or in association (26.8% of cases). All patients used topical steroids at least once in the studied year. Systemic antihistamine therapy was used by 25.6% of patients. In this cohort, 32.7% of patients required two or more examinations per year for exacerbations of their symptoms. Conclusion: Vernal keratoconjunctivitis is a severe ocular condition that mainly affects young males. Vernal keratoconjunctivitis is characterized by different clinical features and therapeutic responses, suggesting the need for a standardized therapeutic approach on the basis of a grading of disease severity.

Anti-68 kDa antibodies in autoimmune sensorineural hearing loss: are these autoantibodies really a diagnostic tool?

Objectives: Autoimmune sensorineural hearing loss (ASNHL) is a relatively rare disorder which can lead to total deafness. At present, no specific laboratory test with adequate sensitivity and specificity is available to confirm the clinical suspicion of ASNHL. The aim of this study was to identify if evaluation of anti-hsp70 antibodies is an accurate diagnostic tool in patients affected by ASNHL. Study design: Prospective study. Methods: During 4-year (2001–2005), all patients with SNHL who were referred to the Eye, Ear, Nose and Throat Department of Parma University, Italy, underwent specific tests to determine the autoimmune origin of the disease. Patients with a consistent suspicion of ASNHL underwent the routine serologic tests and a test for determination of anti-hsp70 antibodies. The same patients were divided into three groups: (1) idiopathic ASNHL; (2) ASNHL associated with ocular inflammation, i.e. Cogans Syndrome; (3) ASNHL associated with a systemic autoimmune disease (SAD). The control group included: (1) healthy subjects; and (2) patients affected by SAD, without any ocular or audiovestibular disease. Results: 88 subjects (67 patients, defined as “study group”, and 21 controls) were evaluated. Anti-hsp70 antibodies were isolated in 52% of the study group patients, and in 4% of the control group (χ2 = 13.009, p < 0.01). In the idiopathic ASNHL patients, 59.5% were found positive for anti-hsp70 antibodies. About 50% of patients affected by CS and 37.5% of patients affected by SAD with SNHL were found positive. In the control group, anti-hsp70 antibodies were found in 8.3% of healthy subjects and in none of the patients with SAD and no hearing loss. Conclusions: The present study confirms the value of the anti-hsp70 test in the serological diagnosis of autoimmune hearing loss. It is still the only available diagnostic marker that identifies an autoimmune origin of hearing loss.

Neuro-Behçet’s disease in childhood: A focus on the neuro-ophthalmological features

Neuro-Behçet’s disease (NBD) involves the central nervous system; peripheral nervous system involvement is not often reported. NBD is quite common in adult patients and occurs rarely during childhood and adolescence. Young patients may share symptoms and signs of NBD with other neuro-ophthalmological disorders (e.g. idiopathic intracranial hypertension); thus, making the differential diagnosis difficult. Neuroimaging is mandatory and necessary for a correct NBD diagnosis but in children radiological examinations are often difficult to perform without sedation. From 1971 to 2011, 130 patients aged ≤16 years have been reported with NBD, according to retrospective surveys, case series, and case reports. The origin of the reported cases met the well-known geographical distribution of Behçet’s disease (BD); the mean age at presentation of neurological findings was 11.8 years, with male gender prevalence (ratio, 2.9:1). We considered in detail the neuro-ophthalmological features of the 53 cases whose neuroimaging alterations were described with an assigned radiological pattern of the disease (parenchymal: 14 cases, non-parechymal: 35 cases, and mixed: 4 cases). In 19/53 patients (36%), neuro-ophthalmological symptoms anticipated any pathognomonic sign for a BD diagnosis, or only occasional aphtae were recalled by the patients. Family history was positive in 17% of subjects. Headache was reported in 75% of the patients; in those presenting with cerebral vascular involvement, headache was combined to other symptoms of intracranial hypertension. Papilledema was the most frequently reported ophthalmological finding, followed by posterior uveitis. Treatment consisted of systemic steroids in 93% of patients, often combined with other immunosuppressive drugs (especially colchicine and azathioprine). Clinical recovery or improvement was documented in the large majority of patients. Nine subjects had definitive alterations, and one died. Based on our review and personal experience, a delayed diagnosis, and the consequently delayed immunosuppressive treatment, may favour permanent sequelae, in particular, optic atrophy.

Low-molecular-weight sodium hyaluronate in the treatment of bacterial corneal ulcers

A double-blind clinical trial was performed on 26 patients suffering from corneal ulcers of proven (i.e., culture-positive) bacterial etiology. After their recruitment, the subjects were randomly assigned to one of the following treatment protocols: (1) tobramycin (15 mg/ml) in saline applied at 1 drop/h or (2) tobramycin (15 mg/ml) in low-molecular-weight hyaluronic acid applied at 1 drop/h. The sample size was adjusted according to a type I error of 0.01 and type all error of 0.05 for a minimal expected difference of 35%. The healing time was calculated from the beginning of treatment to the day on which a follow-up fluorescein test proved to be negative. The mean healing time (±SD) was 3.5 ±0.9 days in the sodium hyaluronate group and 5.9 ± 1.5 days in the saline group (P < 0.001). These results suggest that treatment with an antibiotic dissolved in low-molecular-weight sodium hyaluronate can further shorten the clinical course of a bacterial corneal ulcer.

Autoimmune uveitis in children: clinical correlation between antinuclear antibody positivity and ocular recurrences.

Abstract.Objective. The aim of this study was to identify the correlation between antinuclear antibody (ANA) titre and the onset and clinical course of uveitis in children with juvenile idiopathic arthritis (JIA) or without any other systemic autoimmune disease, i.e., idiopathic uveitis (IU). Methods. Twenty-two patients affected by uveitis were examined. Ten had JIA-associated uveitis, 12 had IU. Follow-up ranged from 7 to 101 months. The ANA were titrated three times per year and additionally in case of ocular recurrences. All patients were treated with immunosuppressive drug combination therapy (IDCT). Results. JIA-associated uveitis: ocular recurrences were noted in three ANA-positive patients and in one ANA-negative patient. IU uveitis: ocular recurrences were noted in one ANA-positive and in one ANA-negative patient. No significant rise in ANA titre was noted in either group during uveitis recurrence. Conclusions. (1) ANA had no value in predicting the recurrence of uveitis. (2) IDCT does not influence ANA production.

Partial third nerve palsy after Measles Mumps Rubella vaccination

BackgroundMeasles Mumps Rubella (MMR) vaccination is known to cause some serious adverse events, such as fever, rash, gland inflammation and neurologic disorders. These include third and sixth cranial nerve palsies.ResultsThe case reported describes a partial recurrent oculomotor palsy associated with systemic symptoms following MMR vaccination in a healthy young child. The oculomotor palsy did not recover completely during the follow-up.ConclusionsMost of the times, measles, mumps and rubella cause mild illness and discomfort; but can also have serious or fatal sequelae. MMR vaccination has been proved to be safe and to reduce significantly the number of reported infections due to these viruses. However, significant adverse events can occur and paediatricians and public health operators should be aware of this aspect.

Perioperative prophylaxis to prevent recurrence following cataract surgery in uveitic patients: a two-centre, prospective, randomized trial.

To compare the postoperative risk of inflammatory relapse in two groups of uveitic patients who underwent cataract surgery: one group had perioperative topical steroids alone and the other used topical and oral steroids.

Syphilitic interstitial keratitis: treatment with immunosuppressive drug combination therapy.

Objective: The following is a case presentation of congenital syphilitic keratitis in a boy 6 years of age who was successfully treated with an immunosuppressive drug combination therapy. Methods: Congenital syphilitic keratitis was diagnosed by clinical findings and laboratory tests. The child was unresponsive to traditional treatment; thus, systemic immunosuppressive therapy, which consisted of oral cyclosporine 4 mg/kg/d, 6 days per week, and oral low-dose steroids (fluocortolone 0.8 mg/kg a week, given every other day), was initiated. Results: Corneal disease showed great improvement with this therapy, with progressive healing of lesions in the first month of treatment and no signs of toxic renal, hepatic, or growth abnormalities. Recurrences of uveitis have not occurred, and corneal interstitial keratitis episodes have been limited to 3 in an 8-year period. After 6 months with no recurrences, a tapering off of the systemic therapy was initiated, and the child is still asymptomatic and without flare-ups. Conclusions: Congenital syphilitic keratitis is usually treated with topical steroids and cycloplegic drugs, which not only can be ineffective but can also lead to complications such as cataract and glaucoma. In the present case report, a pediatric patient affected by syphilitic interstitial keratitis was treated successfully with an immunosuppressive drug combination therapy.