Amit Abraham& Dr LF

13 th May 2010

Consult Service/Walk Rounds

TJ, 20 yo male in good state of healthDizziness, hearing loss & eye irritation

HPI

18th April 2010Tilting of the room towards the leftNoticed upon waking up from bedCouldn’t standWorsened the longer he stood Associated with ringing in left ear (high

pitch)Severe nausea + vomitingNon projectile, consisting of whatever he ate

within minutes

Admitted to HospitalWorked up for Multiple SclerosisDiagnosed as having LabrynthitisGiven Antivert, which didn’t helpTried a herbal concoction, which did helpOutpatient audiogram (20th April) mild

decrease in hearing in left ear

Over 1.5 weeks, noticed gradual loss of hearing in left ear, in addition to the ringing

Dizziness was still present

On 30th April 2010 (Friday), mom noticed that left eye was inflamed and red

No pain, tenderness, tearing or visual changes

Disappeared after 1 day

Over the next week, noticed ringing in right ear, with gradual hearing loss

Reports easy fatigabilityOn 7th May 2010, he suddenly developed

bilateral eye redness, pain, lacrimation and photosensitivity

Dizziness worsened and had trouble walking

Admitted to HospitalOphthalmology concerned about uveitis (no

slit lamp available) and recommended cyclopentolate and prednisolone eye drops

ENT concerned about Cogan’s and recommended 60mg prednisolone

LP: WBC:0 RBC: 8 Protein: 40 Glc: 59ESR: 15 CRP: 0.9 C3: 125.5 C4: 23.5Urinanalysis –ve Tox screen –ve

HPI

Denied fever, night sweats, chillsNo myalgias, arthralgias, vomiting, diarrhea,

constipationNo new rashes, ulcers, photosensitivityNo chest pains, shortness of breathNo convulsionsNo hx of travel, sick contacts

PMHx

Hemorrhoids (14th April) – proctosol 2% TIDMeds: HerbalNo OTC, vitamin supplementsAllergies: metal PSH: none

Family Hx

Mother – MS, diagnosed at 16 on IVIgPaternal aunt – MS diagnosed at 14, died of

complicationsMaternal Grandmother – SLE, diagnosed at

75Paternal Grandmother with IBD, died of colon

ca12 yo brother with CF

SHx

Lives in QueensPart time job in cateringGoes to Queensboro Community College

(liberal arts)15 cigarettes/day x 5 yrsNo alcoholOccasional marijuana

Exam

T: 36.9 BP: 129/67 P: 95 RR: 20Gen: NAD. Multiple tattoos on both armsHEENT: no hearing in left ear, partial hearing

in right. Able to carry a normal conversation. EOMI. No nystagmus. No erythema. No ulcers. Intact gag. Uvula midline

Neck: midline trachea, no lymphadenopathy

Chest: hyperpigmented macular rash (molluscum contagiosum). Clear to auscultation

CVS: Regular rate and rhythym. No murmurs or gallops

Abd: erythema in area of belt buckle, + BS, no hepatosplenomegaly

Extremities: Folliculitis on left shin, intact peripheral pulses

Labs

WBC: 14 (PMNs: 94%) Hgb: 13.3 Hct: 40.5 Plt 262 139 106 10 150 4.2 23 0.8AST: 19 ALT: 10 ALP: 53 Tot Bili: 0.4Tot Prot: 7.4 Alb: 3.6

DDx

Cogan’s SyndromeInfection: Syphilis, TB, Lyme, Chlamydia,

ViralRheumatologic: Wegner’s Granulomatosis,

RA, Sjogren’s, SLE, PAN, TakayasuSarcoid, Meniere’s, IBD,

Cogan’s Syndrome

Chronic inflammatory disease of young adults

Involves ocular and audiovestibular organs

Clinical Diagnosis

Etiology & Pathogenesis

Prodrome of URI in 25% of pts

Chlamydia, Borrelia, Treponema, CMV, HSV, Parvovirus, Hepatitis

No conclusive evidence

Lymphocytic and plasma cell infiltrationEndolymphatic hydropsDegenerative changes in the organ of CortiExtensive new bone formation in the inner

earDemyelination & atrophy of CN VIII

Animal models demonstrate that CD4+Tcells instrumental in pathology

Antigens such HSP 70 and those sharing sequence homology with Ro and reovirus III major core protein lambda I in few cases

Ocular Manifestations

Non Syphilitic interstitial keratitisInflammation of corneal stromaCellular infiltration & neovascularizationUsually non ulcerativeIrregular, granular infiltratePain, photophobia, lacrimation

Other ocular complications:Conjunctivitis IritisScleritisEpiscleritisPosterior uveitisRetinal vasculitisVitreitis

Audiovestibular Manifestations

Sudden unilateral hearing reduction with tinnitus, with gradual bilateral sensorineural loss

Repeated episodes leads irreversible deafness over a short period of time

Vertigo, ataxiaNausea, vomiting

Systemic Manifestions (20% - 66%):

Constitutional symptomsNecrotising vasculitis affecting aorta, renal,

mesenteric, iliac vesselsAortic valve diseaseGINeurologic Skin & mucous membrane

Diagnosis

Slit lamp examinations to diagnose the ocular disease

Formal Audiometry

Serial studies every 6 months to assess progress of disease

ESR, CRP, CBC, CreatinineANA, ENA, RF, complememnt, ANCAVRDL/RPR, FTA-ABS, Lyme, TBEBV, HSV< CMV, MumpsCT/MRI

Therapy

Eye disease is easier to manageTopical glucocorticoid eyedropsRarely, cyclosporine eyedropsIf eye involvement is not IK, need systemic

corticosteroids1mg/kg/day with tapering after 2-4 weeks

Inner ear disease requires high dose prednisolone for 2 weeks followed by taper

May last 4-6monthsRepeated flares can cause deafnessCochlear implantsVertiginous symptoms treated with

antihistamines (meclizine 25-50mg q6h) or benzodiazepines (diazepam 2-10mg q6h)

Consider steroid sparing agents for repeated flares

MethotrexateCyclosporine