Sensorineural Hearing Loss Arthritis Episcleritis

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The diagnosis of CS is based on the presence of both ocular inflammation and vestibulo-auditory dysfunction. IK and other anterior ocular segment problems are diagnosed by slit-lamp examination, while posterior ocular segment disorders are recognized by ophthalmo-scopy. Retinal vascular abnormalities may be revealed using fluorescein angiography.

Vestibuloauditory disease may result from a toxic/metabolic disturbance or a peripheral (labyrinth, vestibule, or cochlea) or central (brainstem, cerebellum, or cerebral cortex) lesion in the nervous system. The vestibuloauditory features of CS are due to a peripheral lesion, namely, in the inner ear. The examination should include a careful examination of the ear canal and tympanic membrane, a hearing test, and a neurological evaluation. In CS, nystagmus is only observed during the acute attack.

Several different tests can be used to diagnose vestibular dysfunction in the appropriate clinical setting. Electronystagmography (ENG) is the most useful and can identify vestibular asymmetry from various causes of vestibular neuronitis. If uncertainty about the location of the lesion exists after ENG, rotary chair testing can be performed to evaluate vestibular function. The hearing loss of CS is sensorineural in origin and comes from damage to the cochlear structures. These patients should undergo pure tone and speech audiometry to document the extent of hearing loss. In CS, the loss of hearing occurs preferentially at the low and high frequencies. Brain-stem auditory-evoked potentials (BAEPs) may be useful in difficult cases. A delay in wave I of the BAEP points toward a cochlear lesion and is compatible with the diagnosis of CS. Demyelinating diseases such as MS and cerebellopontine tumors also cause hearing loss and vertigo, and are excluded by magnetic resonance imaging (MRI) of the brain using gadolinium contrast.

Patients with systemic complaints should undergo further evaluation, depending on the nature of their symptoms or findings. Symptoms such as dyspnea, chest pain, or an abnormal heart examination (e.g., diastolic heart murmur) warrant a thorough cardiac examination, which may include chest X ray, electrocardiogram, echocardiogram, cardiac MRI scan, or coronary angiography. These studies may reveal aortic insufficiency, aortitis, or sites of coronary occlusion from ostial disease or arteritis. Bruits heard over a large peripheral artery suggest the presence of a large-sized vessel vasculitis and may be an indication for magnetic resonance angiography (MRA) or conventional angiography. Smooth tapered stenoses, dilatations, and aneurysms can be seen in large- and medium-sized vessel vasculitis.

The differential diagnosis of CS is broad and includes a diverse group of conditions with ocular and vestibuloauditory pathology, as well as systemic manifestations. These disorders are summarized in Table 3. Reliable assessment of disease in multiple organ systems is essential for appropriate management of CS and often requires a team of specialists, including ophthalmologists, otolaryngologists, audiologists, rheumatologists, and cardiologists.

TABLE 3 Differential Diagnosis of Cogan's Syndrome

Disorder

Eye manifestations

Ear manifestations

Other features

Chlamydia infection

Conjunctivitis, IK

Otitis media, CHL

Respiratory tract symptoms

Lyme disease

Conjunctivitis, episcleritis, uveitis, IK,

-

Erythema, migraines, meningitis, carditis, arthritis

choroiditis, retinitis, optic neuritis

Congenital syphilis

IK

SNHL

+FTA-ABS

Whipple's disease

Uveitis, vitritis

SNHL

Diarrhea, weight loss, fever, arthritis, skin hyperpigmentation

Sarcoidosis

Conjunctivitis, IK, anterior uveitis, retinitis,

SNHL

Hilar adenopathy, pulmonary fibrosis, CNS involvement, skin lesions,

keratoconjunctivitis sicca

parotid gland enlargement

Vogt-Koyanagi-Harada

Panuveitis, iridocyclitis

Vertigo, SNHL

Aseptic meningitis, vitiligo, alopecia, poliosis

KID syndrome (congenital)

Keratoconjunctivitis, corneal

SNHL

Ichthyosis

vascularization

Sjogren's syndrome

Keratoconjunctivitis sicca

SNHL

Xerostomia, parotid gland enlargement, serum ANA

Rheumatoid arthritis

Episcleritis, scleritis

SNHL

Arthritis, serum rheumatoid factor

SLE

Retinitis, optic atrophy

SNHL (mild)

Skin rash, arthritis, pleurisy, glomerulonephritis, cytopenias,

serum ANA

APA

Retinal vascular occlusion

SNHL

Deep vein thrombosis, pulmonary emboli, arterial thrombosis,

thrombocytopenia, serum APA

Polyarteritis nodosa

Retinal vasculitis

SNHL

Renal failure, hypertension, arthritis, skin lesions, neuropathy, CNS

involvement, elevated ESR

Wegener's granulomatosis

Conjunctivitis, episcleritis scleritis,

Otitis media (CHL),

Sinusitis, pulmonary infiltrates, glomerulonephritis, serum ANCA

uveitis, retinitis

SNHL

Relapsing polychondritis

Conjunctivitis, IK, scleritis, uveitis

SNHL

Auricular, nasal, and laryngotracheal chondritis, systemic vasculitis

Behget's syndrome

Anterior uveitis, episcleritis, IK, retinal

Vertigo, SNHL

Oral and genital ulcers, CNS involvement, arthritis, skin lesions

vasculitis, chorioretinitis

Ulcerative colitis

Anterior uveitis

SNHL

Colitis

Crohn's disease

Anterior uveitis

SNHL

Enterocolitis

CNS lymphoma

Corneal, anterior chamber and vitreous

SNHL

Cerebellopontine mass

opacities, sub-RPE infiltrates

CLL

Optic neuropathy

Otitis media, SNHL

CNS involvement, CSF lymphocytosis

Retinocochleocerebral

Retinal arteriolar occlusions

SNHL

CNS microangiopathy

vasculopathy

Abbreviations-. ANCA antineutrophil cytoplasmic antibodies; APA, antiphospholipid antibody; ANA, antinuclear antibodies; CHL, conductive hearing loss; CLL, chronic lymphocytic leukemia; CNS, central nervous system; CSF, cerebrospinal fluid; FTA-ABS, fluorescent treponemal antibody absorption; IK, interstitial keratitis; KID, keratitis, ichthyosis, and deafness; RPE, retinal pigment 01 epithelial; SLE, systemic lupus erythematosus; SNHL sensorineural hearing loss.

Abbreviations-. ANCA antineutrophil cytoplasmic antibodies; APA, antiphospholipid antibody; ANA, antinuclear antibodies; CHL, conductive hearing loss; CLL, chronic lymphocytic leukemia; CNS, central nervous system; CSF, cerebrospinal fluid; FTA-ABS, fluorescent treponemal antibody absorption; IK, interstitial keratitis; KID, keratitis, ichthyosis, and deafness; RPE, retinal pigment 01 epithelial; SLE, systemic lupus erythematosus; SNHL sensorineural hearing loss.

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