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 Table of Contents    
CASE REPORT
Year : 2017  |  Volume : 10  |  Issue : 3  |  Page : 232-234  

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


Department of Ophthalmology, Leicester Royal Infirmary, Leicester, UK

Date of Web Publication5-Oct-2017

Correspondence Address:
Konstantinos T Tsaousis
Department of Ophthalmology, Medical Retina, Leicester Royal Infirmary, Infirmary Square, Leicester, Leicestershire, LE1 5WW
UK
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ojo.OJO_170_2014

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   Abstract 

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.

Keywords: Deafness, human leukocyte antigen-A2, sympathetic ophthalmia


How to cite this article:
Tsaousis KT, Konidaris VE, Empeslidis T. Bilateral sensorineural hearing loss secondary to sympathetic ophthalmia in a human leukocyte antigen-A2 positive patient. Oman J Ophthalmol 2017;10:232-4

How to cite this URL:
Tsaousis KT, Konidaris VE, Empeslidis T. Bilateral sensorineural hearing loss secondary to sympathetic ophthalmia in a human leukocyte antigen-A2 positive patient. Oman J Ophthalmol [serial online] 2017 [cited 2019 Aug 24];10:232-4. Available from: http://www.ojoonline.org/text.asp?2017/10/3/232/215994


   Introduction Top


The overlap of features among sympathetic ophthalmia and Vogt-Koyanagi-Harada (VKH) syndrome have been reported by numerous researchers.[1],[2],[3] 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.[4],[5] Comer et al. and Nirankari et al. have reported the coexistence of sympathetic ophthalmia and neurosensory deafness.[6],[7] 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 Top


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 1]a. 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

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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 1]b.
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

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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 Top


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 Top


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.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
   References Top

1.
Davis JL, Mittal KK, Freidlin V, Mellow SR, Optican DC, Palestine AG, et al. HLA associations and ancestry in Vogt-Koyanagi-Harada disease and sympathetic ophthalmia. Ophthalmology 1990;97:1137-42.  Back to cited text no. 1
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2.
Al-Halafi A, Dhibi HA, Hamade IH, Bou Chacra CT, Tabbara KF. The association of systemic disorders with Vogt-Koyanagi-Harada and sympathetic ophthalmia. Graefes Arch Clin Exp Ophthalmol 2011;249:1229-33.  Back to cited text no. 2
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3.
Chuang CT, Huang PS, Chen SC, Sheu SJ. Reversible alopecia in Vogt-Koyanagi-Harada disease and sympathetic ophthalmia. J Ophthalmic Inflamm Infect 2013;3:41.  Back to cited text no. 3
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4.
Chuah SY, Lyne AJ, Dronfield MW. Vogt-Koyanagi-Harada syndrome, a rare association of Hodgkin's disease. Postgrad Med J 1991;67:476-8.  Back to cited text no. 4
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5.
Arevalo JF, Garcia RA, Al-Dhibi HA, Sanchez JG, Suarez-Tata L. Update on sympathetic ophthalmia. Middle East Afr J Ophthalmol 2012;19:13-21.  Back to cited text no. 5
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6.
Comer M, Taylor C, Chen S, Martin K, Jordan K, Meyer P. Sympathetic ophthalmia associated with high frequent deafness. Br J Ophthalmol 2001;85:496.  Back to cited text no. 6
    
7.
Nirankari MS, Khanna KK, Chawla GD, Mathur RP. Sympathetic ophthalmitis with total deafness (a case report). J All India Ophthalmol Soc 1970;18:29-32.  Back to cited text no. 7
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8.
Sugita S, Sagawa K, Mochizuki M, Shichijo S, Itoh K. Melanocyte lysis by cytotoxic T lymphocytes recognizing the MART-1 melanoma antigen in HLA-A2 patients with Vogt-Koyanagi-Harada disease. Int Immunol 1996;8:799-803.  Back to cited text no. 8
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9.
Reynard M, Shulman IA, Azen SP, Minckler DS. Histocompatibility antigens in sympathetic ophthalmia. Am J Ophthalmol 1983;95:216-21.  Back to cited text no. 9
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    Figures

  [Figure 1], [Figure 2]



 

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