OTOLOGY

Wolfgang Wagner Æ Astrid Fehrmann

Association of retinal vasculitis (Eales’ disease) and Meniere-likevestibulocochlear symptoms

Received: 27 January 2005 / Accepted: 9 March 2005 / Published online: 24 August 2005� Springer-Verlag 2005

Abstract The objective of this study was to present apreviously unknown association of visual loss andMeniere-like vestibuloauditory symptoms in Eales’ dis-ease (idiopathic retinal vasculitis) and to give a survey ofother conditions affecting both the retina and inner ear.A 32-year-old man diagnosed with Eales’ disease pre-sented with recurrent deteriorations of visual acuity andsimultaneous recurrent vestibuloauditory symptomsstrongly reminiscent of Meniere’s disease. Both therecurrent low frequency hearing losses and the losses ofvisual acuity could be restituted repeatedly by high dosei.v. corticosteroids. A literature review reveals that in anumber of other retinopathic diseases associated withMeniere-like symptoms, microvasculopathy is also thepathomechanism. A common pathogenetic cause, e.g.,autoimmune-mediated microvasculitis of retinal peri-phlebitis and vestibulocochlear symptoms, in this case issuggested. The presented case adds new evidence tomicrovasculopathy being a pathogenetic factor inMeniere’s disease. In cases of coincidence of vestibulo-cochlear symptoms and retinal perivasculitis, a thera-peutic attempt with high-dose corticosteroids isadvisable.

Keywords Retinal vasculitis Æ Eales’ disease ÆMeniere’s disease Æ Hearing loss

Introduction

Eales’ disease is characterized by retinal occlusivevasculitis and perivasculitis, peripheral capillary non-perfusion and retinal neovascularization (Fig. 1).Mainly young men are affected. For unknown reasons,Eales’ disease is rare in developed countries andespecially common in the Indian subcontinent, with areported incidence in India of 1 in 200 to 250 oph-thalmologic patients [24]. The etiology is unknown.Favorable response to systemic steroids, lymphocyticinfiltration in a histopathologic study of the vitreousand epiretinal membranes and abnormal immunologi-cal parameters observed in retinal vasculitis similar toEales’ retinopathy all indicate an immunologicalmechanism. However, immunological studies (e.g.,serum immunoglobulin levels, level of circulatingimmune complexes and T-lymphocyte subsets) failedto present definite evidence for this hypothesis [7].Also discussed, but not proven, is tuberculosis as anetiological agent. The natural course of the conditionranges from spontaneous remission to continuousprogression and eventual loss of vision. Therapy issymptomatic and comprises delivery of corticosteroids,laser photocoagulation and cryotherapy of the retina,or vitrectomy.

In 1983 Renie et al. [25] reported a high prevalence ofunexplained sensorineural hearing loss and vestibulardysfunction in a group of patients with Eales’ disease.The authors suggested a common pathogenetic mecha-nism of retinal periplebitis and vestibulocochlear symp-toms, e.g., simultaneous vasculopathic affection of theeye and ear.

Here, the first case of an association of ophthalmo-logic and Meniere-like vestibulocochlear symptoms in apatient with Eales’ disesae is reported. The identical timecourse of the ophthalmologic and auditory symptoms,including parallel positive response to systemic medicaltreatment, suggests a shared pathogenetic mechanism inthis case.

W. Wagner (&)Department of Otorhinolaryngology andHead and Neck Surgery, University of Tuebingen,Elfriede-Aulhorn-Str. 5, 72076 Tuebingen, GermanyE-mail: w.wagner@med.uni-tuebingen.deTel.: +49-7071-2988088Fax: +49-295674

A. FehrmannDepartment of Ophthalmology, Katharinen-Hospital, Stuttgart,Germany

Eur Arch Otorhinolaryngol (2006) 263: 100–104DOI 10.1007/s00405-005-0967-5

Case report

A 32-year-old man complained of progressive bilateralhearing impairment, tinnitus and occasional attacks ofwhirling vertigo with nausea that had been going on for3 years. At the same time, he had also noticed loss ofvisual acuity, and the diagnosis of idiopathic retinalperivasculitis (Eales’ disease) was established. For thishe had undergone repeated surgery in both eyes, e.g.,laser and cryo-coagulation of the retina and vitrectomieswith implantation of artificial lenses. As to the vestibu-locochlear symptoms, treatment by different doctors hadconsisted of rheologic infusions, oral intake of betahis-tidine, iontopheresis and retroauricular subcutaneousinjection of meaverine. One month before presentationin our clinic, he had suffered a documented completeloss of his right vestibular organ, which had restituted toalmost normal function again. Past medical history re-vealed a negative borreliosis titer (controlled twice),unremarkable findings in a general medical examinationincluding complete blood count, an unremarkable neu-rological examination including Doppler sonography ofthe extracranial cerebral arteries and a normal MRI ofthe central nervous system.

Actual pure tone audiometry in our clinic showedbilateral sensorineural hearing loss with thresholdmaxima around 1 kHz (see Fig. 2a). Ear microcopy wasnormal. Evoked-response audiometry was normal onthe right side, while no significant potentials could beobserved on the left. Examination of the vestibularsystem including caloric testing demonstrated slightlydecreased, but still normal function on both sides.Transiently evoked otoacoustic emissions were dimin-ished on the left side, while they were preserved on theright side in the low frequency range. In the actualophthalmologic examination, visual acuity was 0.5 on

the right and 0.4 on the left (with correction). Serologicalimmunopathologic analysis revealed a discrete presenceof anti-phospolipide antibodies of the IgM-type; im-muno-electrophoresis was unremarkable. These twofindings were interpreted as compatible with an auto-immune disease, but were not highly typical for such acondition.

The patient was put on an i.v. corticosteroid regimen(day 1 and 2:250 mg prednisone in 500 ml dextrane i.v.over 4 h, day 3 and 4:150 mg prednisone i.v.; day 5 and6:100 mg i.v.; day 7:75 mg i.v.; day 8:50 mg i.v.; day 9and following: further tapering and oral delivery).Within 2 days after onset of this therapy, the hearingloss improved significantly (pantonal improvement by20 to 30 dB on the right and 10 to 20 dB on the left side;see Fig. 2b) and, unexpectedly, also vision improvedsubjectively and objectively (rise of visual acuity to 0.7on the right and 0.6 on the left). When the dose wasrestricted to 5 mg prednisone/day 6 weeks after begin-ning the therapy, the bilateral hearing and vision dete-riorated again synchronously. The steroid dose wasraised to 10 mg/day per os with no positive effect for6 days. Then the high dose i.v. steroid regimen wasstarted again, and within 6 days hearing and visionre-improved to the better status as described above. Inthe following, both hearing and vision remained stableunder a steroid dose of 20 mg prednisolone per os for atleast 3 months, when follow-up was ended by thepatient.

Discussion

There is only one literature reference reporting signifi-cant coincidence of Eales’ disease and vestibulocochlearsymptoms [24]; however, all studies on Eales’ diseaseoriginate from ophthalmologic institutes, so the focus

Fig. 1 Typical retinal findingsin Eales’ disease (from: [13],with friendly permission). Thickarrow: papilla of optic nerve;thin arrow: macula; solid circle:obliterated retinal vessels;dotted circle: collateral vessels(due to capillary non-perfusion)

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was mostly on ophthalmologic and not on otolaryngo-logical manifestations. Renie et al. [24] reported unex-plained sensorineural hearing loss when compared withthe expected age-related hearing impairment in 6/25patients (24%) with Eales’ disease. In five of these cases,hearing loss was at the frequency 4 kHz and higher; inone case hearing impairment was pantonal. In this re-gard, the hearing loss pattern was different from theaudiometric finding in our case. In 10/23 patients (43%)of the same group, ‘‘markedly’’ or ‘‘moderately’’abnormal vestibular dysfunction was found. This inter-pretation was based on ‘‘caloric tests and tests forspontaneous and positional nystagmus.’’ Unfortunately,further description of the dysfunction and to what extentthe patients were symptomatic is not mentioned in thearticle. The authors suggested a common pathogeneticmechanism of the retinal perivasculitis and vestibulo-cochlear symptoms, e.g., simultaneous vasculopathicaffection of the eye and ear.

One differential diagnosis in the case presented here isthe accidental coincidence of Eales’ disease and Meni-ere’s disease with no pathogenetic association. However,the strict simultaneity in clinical course and response totherapy of the ocular and vestibulocochlear symptomsmake this view highly unlikely.

Although the symptoms in the presented case werenot in all aspects entirely typical for ‘‘classical’’ Meni-ere’s disease—e.g., Meniere’s disease does rarely presentwith simultaneous bilateral acute hearing loss as part ofthe initial symptoms—the striking similarity between thesymptoms presented here and Meniere’s disease maysuggest a common or at least related pathogenesis.Interestingly, a number of other conditions character-ized by ocular microvasculopathy, as is the case in Eales’disease, also include Meniere-like symptoms. Forexample, in Susac syndrome (microvasculopathy withsmall infarctions of retinal, cochlear and encephalic tis-sue in young women), the inner ear symptoms consist ofsensorineural hearing loss, which is typically located inthe low frequencies [29], and atactic gait or whirlingvertigo with nausea [22]. The observation of low-fre-quency hearing loss prompted the hypothesis that aninfarction of the lower cochlear apex might be theunderlying cause [18]. Another example is Cogan’ssyndrome (microvasculopathic inflammatory eye diseaseassociated with inner ear symptoms, with possible sys-temic vasculitic involvement [5]), which also comprisesvestibuloauditory findings strongly resembling Meni-ere’s disease [19, 35, 9]. These examples support thehypothesis of a vasculopathic cause in Meniere’s disease,

Fig. 2 a Pure tone audiogrambefore therapy. b Pure toneaudiogram after 2 days oftherapy (high dose ivcorticosteroids, see text)

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which has been among the numerous suspected etiolo-gies [28].

A previous report of ophthalmologic examinations in31 patients with sudden hearing loss [8] suggested thatshared vascular pathology in the eye and ear mightoccur more often than commonly assumed: Refraction,vision, intraocular pressure and excavation of the opticnerve were normal in all patients. However, in 16 pa-tients (53%) a slight tortuosity of arterial retinal vesselswas found, and 25 patients (81%) displayed abnormalconjunctival vascular ectasia, indicating a disturbedmicrocirculation. As Glacet-Bernard et al. [11] stated intheir case report of central retinal vein occlusion andsudden hearing loss, ‘‘cochlear and retinal blood flowshow some features in common, such as terminalmicrocirculation with a tight-junction endothelium, andan internal pressure (of aqueous or endolymph), whichmay result in limited adaptation to hemodynamic vari-ations.’’

Unlike the inner ear, the eye is amenable to directmorphologic examination and visualization of patho-mechanisms, e.g., retinal vascular processes. Therefore,in cases with strong evidence for a common ocular andvestibulocochlear pathogenesis, the eye provides adiagnostic window, albeit indirect, for investigation ofthe pathological processes in the inner ear. A survey ofsyndromes including both retinopathic changes andvestibuloauditory symptoms is given in Table 1.

Conclusion

A case of recurrent loss of vision in Eales’ disease (ret-inal perivasculitis) associated with vestibulocochlearsymptoms reminiscent of Meniere’s disease is reported.The parallel clinical course of ocular and otologicsymptoms, including simultaneous therapeutic responseto corticosteroids, suggests a common pathogeneticmechanism that is suspected to be microvasculopathy.This case together with other previously described syn-dromes comprising ocular microvasculopathy andMeniere-like symptoms may support the hypothesis of

microvasculopathy being a pathogenetic factor inMeniere-like vestibulocochlear disturbances.

Practical conclusions are that patients with retinalvasculopathy should be seen by an otolaryngologist and,on the other hand, patients complaining of Meniere-likesymptoms should undergo ophthalmologic examination.In patients with an association of vestibulocochlearsymptoms and retinal vasculopathy, a therapeutic effortwith high-dose i.v. corticosteroids is advisable.

References

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Table 1 Known syndromes with retinopathy and vestibulocochlear symptoms

Etiology Name of syndrome/disease

Genetic disorder Alport, Alstrom, Bardet-Biedl (congenital hypogonadism), Bassen-Kornzweig, Cockayne, cystinuria,Davenport, Diallinas-Amalric, Edward, facsioscapulohumeral muscular dystrophy, Flynn-Aird,Forsius-Eriksson, Friedreich, Gordon-Capute-Konigsmark, Hallervorden-Spatz, Hallgren, Hersch,Hunter (mucopoly-saccharidosis), Kartagener, Kearns-Sayre, Leber, Norrie, olivopontocerebellaratrophy type III, Pfeiffer-Junemann-Polster-Bauer, Pierre-Marie, pipecoline acidemia, Refsum,Reinstein-Chaflin, Shy-Gonatas, Stickler, Usher, Werner, Young-Leon-Barth-Green,Zellweger [2, 3, 10, 15, 14, 17, 26, 30]

Infectious etiology Rubella (intrauterine infection) [21]Autoimmune vasculitis Behcet [20, 23]; Wegener [16, 32]; polyarteritis nodosa [6, 34]; Giant cell arteritis (Horton) [1, 27];

lupus erythomatodes [1, 32]Other etiology Relapsing polychondritis (inflammatory reaction in multiple cartilages, autoimmune mechanism?) [4, 12]Uncertain etiology Susac [18, 22, 29, 33]; Cogan1 (autoimmune mechanism?) [5, 9]; Takayasu (autoimmune mechanism?) [31]

1in the so-called typical Cogan’s syndrome where the classical ophthalmologic finding is interstitial keratitis, but retinal pathology (retinalartery occlusion, retinal detachment) can also occur [7]

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14. Kamphorst W, Haasnot KJP, Wegener RP, Dorsman WH(1995) A congenital syndrome of mental deficiency, gait dis-turbance, sensorineural deafness and pigmentary retinopathyassociated with premature atherosclerosis. Clin Neuropathol14:211–215

15. Konigsmark BW, Gorlin RJ (1976) Genetic and metabolicdeafness. Saunders, Philadelphia

16. Lamprecht P, Lerin-Lozano C, Reinhold-Keller E, Nolle B,Gross WL (2000) Retinal artery occllusion in Wegener’sgranulomatosis. Rheumatology (Oxford) 39:928–929

17. Leiber B (1990) Die klinischen Syndrome, 7th edn. Urban undSchwarzenberg, Munich

18. MacFayden DJ, Schneider RJ, Chisholm IA (1987) A syn-drome of brain, inner ear and retinal microangiopathy. CanJ Neurol Sci 14:315–318

19. McDonald TJ, Vollertsen RS, Younge BR (1985) Cogan’ssyndrome: audiovestibular involvement and prognosis in 18patients. Laryngoscope 95:650–654

20. Ouazzani B, el Aouni A, Hajji Z, Benchekroun N, Chaoui Z,Boulanounar A, Benchrifa F, Agnaou L, Berraho-Hammani A(1996) Retinal vasculitis. J Fr Ophtalmol 19:8–12

21. Parving A, Starup K (1977) Audiometric and ophthalmologicalfindings in rubella deafness. Audiology 16:49–54

22. Petty GW, Matterson EL, Younge BR, McDonald TJ, WoodCP (2001) Recurrence of Susuc syndrome (retinocochleocere-bral vasculopathy) after remission of 18 years. Mayo Clin Proc76:958–960

23. Pollak L, Luxon LM, Haskard DO (2001) Labyrinthineinvolvement in Behcet’s syndrome. J Laryngol Otol 115:522–529

24. Putamma ST (1970) Varied fundus picture of central retinalvasculitis. Trans Asia Pacific Acad Ophthalmol 3:520

25. Renie WA, Murphy RP, Anderson KC, Lippman SM,McKusick VA, Proctor LR, Shimizu H, Patz A, Fine S (1983)The evaluation of patients with Eales’ disease. Retina 4:243–248

26. Russell-Eggitt IM, Clayton PT, Coffey R, Kriss A, Taylor DSI,Taylor JFN (1998) Alstrom syndrome. Ophthalmology105:1274–1280

27. Saadoun D, Cacoub P, Costedoat-Chalumeau N, Sbai A, PietteJC (2000) Horton’s disease and corticosteroid-responsivehearing loss. Ann Med Interne 151:600–602

28. Schuknecht HF (1974) Pathology of the ear. Harvard Univer-sity Press, Cambridge, pp 453–465

29. Schwitter J, Agosti R, Ott P, Kalman A, Waespe W (1992)Small infarctions of cochlear, retinal and encephalic tissue inyoung women. Stroke 23:903–907

30. Shastry BS, Hiraoka M, Trese DC, Trese MT (1999) Norriedisease and exudative vitreo-retinopathy in families withaffected female carriers. Eur J Ophthalmol 9:238–242

31. Siglock TJ, Brookler KH (1987) Sensorineural hearing lossassociated with Takayasu’s disease. Laryngoscope 97:797–800

32. Stone JH, Francis HW (2000) Immune-mediated inner eardisease. Curr Opin Rheumatol 12:32–40

33. Susac JO (1994) Susac’s syndrome: the triad of microangiop-athy of the brain and retina with hearing loss in young women.Neurology 44:591–593

34. Tsunoda K, Akaogi J, Ohya N, Murofushi T (2001) Sensori-neural hearing loss as the initial manifestation of polyarteritisnodosa. J Laryngol Otol 115:311–312

35. Vollertsen RS, McDonald TJ, Younge BR, Banks PM, StansonAW, Ilstrup DM (1986) Cogan’s syndrome: 18 cases and areview of the literature. Mayo Clin Proc 61:344–361

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