cogan's syndrome - a very rare disorder that i was fortunate to see
TRANSCRIPT
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