Bilateral sensorineural hearing loss secondary to sympathetic ophthalmia in a human leukocyte antigen-A2 positive patient.

We report a case of sympathetic ophthalmia 1 month following trauma in a 71-year-old immunocompetent female patient of Indian origin. The patient was hospitalized with signs and symptoms of meningism, ataxia, and neurosensory deafness. We explore and provide further clinical evidence in supporting the hypothesis of antigen cross-reactivity derived from tissues with common neural crest embryological background such as the uvea and cells of the labyrinth. The patient was human leukocyte antigen-A2 positive and treatment with oral steroids and cyclosporine has managed to have favorable results and control the inflammation.

Introduction

The overlap of features among sympathetic ophthalmia and Vogt-Koyanagi-Harada (VKH) syndrome have been reported by numerous researchers.[123] Both VKH disease and sympathetic ophthalmia are autoimmune systemic inflammatory disorders affecting various organs containing melanocytes. The affected tissues uvea, leptomeninges, melanoblasts, ocular pigments, and auditory labyrinth pigments have a common embryological origin, the neural crest.[45] Comer et al. and Nirankari et al. have reported the coexistence of sympathetic ophthalmia and neurosensory deafness.[67] In Comer's case, the patient was positive for human leukocyte antigen (HLA)-A2, HLA-B51, and HLA-D4. We report a case of sympathetic ophthalmia 1 month following trauma, explore and provide further clinical evidence in supporting the hypothesis of antigen cross-reactivity derived from tissues with common neural crest embryological background such as the uvea and cells of the labyrinth.

Case Report

A 71-year-old immunocompetent female patient of Indian origin suffering from epilepsy and type II diabetes developed signs of blurred vision in the right eye 1 month following left eye closed globe rupture and complete avulsion of iris as a result of the blunt trauma [Figure 1a]. The patient also developed neurological signs of ataxia, confusion, and bilateral total hearing loss. She was admitted to hospital and ophthalmological assessment was requested by the neurologists.

Figure 1

(a) Blunt trauma of the left eye with complete avulsion of iris. (b) Fundus fluorescein angiography (early phase) of the right eye showing multiple leakages

Best-corrected visual acuity at presentation in the right eye was counting fingers and in the left eye 6/12. There was bilateral anterior chamber cellular activity of 3+ in the right and 1+ in the left eye [Figure 2]. Optical coherence tomography revealed a retinal pigment epithelium irregularity while fluorescein angiography showed pinpoint staining in the right eye [Figure 1b].

Figure 2

Optical coherence tomography images at presentation and during the follow up period. At presentation, significant retinal pigment epithelium irregularities were present while restoration of RPE integrity was achieved after treatment and subsequent improvement of visual acuity. In the third column, fundus images are presented, showing similarities of this clinical entity with Vogt-Koyanagi-Harada (VKH) syndrome

A diagnosis of sympathetic ophthalmia was made and the patient was treated with a 3-day course of Methylprednisolone 1g/day followed by a dose of 80 mg/day of Prednisolone tapered to 40 mg/day over a period of two weeks and discontinued in six weeks time. Cyclosporine A 100 mg/day was commenced at that dose and gradually increased to 300mg/day in four weeks. Visual acuity improved to 6/9 in the right eye with the inflammation settling down and OCT examination showed restoration of RPE integrity in both eyes after two months [Figure 2]. The exciting left eye remained clinically stable having visual acuity 6/12 even 6 months after presentation [Figure 2]. The neurological symptoms settled gradually over a period of two months from initiation of steroids. However, the hearing loss did not recover.

Discussion

Similar to Comer's case in our case the exciting eye sustained significant damage on the iris with complete iris avulsion; however, the sympathizing eye did not develop Dalen-Fuchs nodules. Interestingly, the patient is positive to HLA-A2 but negative for other HLA alleles. In a case series presented by Sugita et al., was suggested that sympathetic ophthalmia and VKH disease are autoimmune diseases directed toward the MART-1 antigen in HLA-A2(+) patients.[8] In a study by Reynard et al., HLA-A2 was present in 65% of the patients with histopathologically diagnosed sympathetic ophthalmia and in 50% in patients with clinically presumed sympathetic ophthalmia.[9] Generally, there is a statistically significant increase in the incidence of one particular histocompatibility antigen in the patient population with a specific disease compared with a matched control population.

Conclusion

An association between histocompatibility antigens and sympathetic ophthalmia has significant clinical implications. Sympathetic ophthalmia is a disease difficult to recognize and diagnose and histocompatibility antigens may provide laboratory support to confirm clinically suspected sympathetic ophthalmia. We provide further clinical evidence of sympathetic ophthalmia, and VKH overlap disease spectrum and that patients with positive HLA-A2 developing signs and symptoms of sympathetic ophthalmia or VKH are potentially at higher risk of neurosensory hearing loss.

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Conflicts of interest

There are no conflicts of interest.