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Case Scenario

Case Scenario. Lisa Gagnon, APRN Connecticut Pediatric Otolaryngology 7 th Annual Symposium October 4, 2012. Intial Presentation CC: Otalgia , Otorrhea. 11y/o female presents to ENT clinic….

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Case Scenario

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  1. Case Scenario Lisa Gagnon, APRN Connecticut Pediatric Otolaryngology 7th Annual Symposium October 4, 2012

  2. Intial PresentationCC: Otalgia, Otorrhea 11y/o female presents to ENT clinic…. • Several years of otalgiaassociated with recurrent otorrhea (clear, then malodorous,yellow) • Reported intermittent tinnitus, hearing loss R>L • Multiple antibiotic drops, systemic Rx- no benefit • Denied Vertigo/headache • PMH- recurrent AOM as infant. Lead exposure requiring chelation, eczema. ?Allergies

  3. ENT Exam Auricles- normal, no tenderness w/manipulation EAC’s with medial watery discharge, erythema TM’s intact/clear RX Vosol, then ½ strength vinegar/water Next month: ↑ scaling, erythema of canal- extending into conchalbowl. HT – Bilateral mixed mod-severe HL, nltymps RX - 1/2str vinegar, external ear moisturization changed to steroidcream/moisturization & dermotic CT scan temporal bones ordered

  4. Audiogram & CT Scan

  5. From Itchy clogged ears →Nl 6mo later (next visit) reported hx ear swelling unresponsive to prior treatments prescribed. Exam: minimal EAC wall changes ?fungal RX: Fluconazole-atomized →1 mo later – did not use fluconazole, reported use of steroid cream topically, felt hearing was nl Exam: normal ear exam- repeat audio wnl

  6. Continued ear complaints… • Next few months…. would flare with erythema, itching, pain, dng, some swelling, primarily of distal canal, ears clogged ? Eczema vs. psoriasis component. Rx: repeated cleanouts, dermotic, aquaphor, steroid creams. • Dermatology referral

  7. 6 months later • Had Dermatology Evaluation completed- Looked good that day- “return when flares” ?Psoriasis vs. eczema Variety of creams prescribed (protopic, clobetasone, derma-smoothe) At that visit felt ears were clogged again…. Much of the same exam, findings . Rx steroid cream, return to dermatology, HT

  8. 1 mo later…. Acutely presented to ED….. Dx with cellulitis • Placed on clindamycin and prednisone by ED • ENT Clinic next day – swelling of auricle without significant tenderness or erythema, lobule spared • Canal walls with scaling and mild erythema, TM’s clear

  9. Dermatology work-up • Skin Biopsy (by Dermatology) showed superficial and deep perivascular and interstitial lymphohistiocytic infiltrate and mild spongiosis and parakeratosis, consistent with dermatitis, possibly allergic contact dermatitis • Panel of 50 visiting dermatogists convened • No identified etiology/allergen

  10. 1 month later, another flare • Similar to last flare, seen in ENT clinic • Now reporting headache preceeding episodes & ringing in ears • EAC’s with similar findings- scaling/erythema, debris • Bilateral SNHL (mod-severe) • High dose steroid Rx • Labs- CBC, ANA, ESR • Rheumatology referral

  11. Audio post treatment

  12. Rheumatology • Labs- CBC, Metabolic, LFT’s, ESR, Thyroid, muscle function, ANA, DNA, ENA screen, complement, IgG, IgA, rheumatoid factor, anti-CCP, CRP, ANCA • CXR- Wnl • Echo- Wnl

  13. Diagnosis….. Relapsing Polychondritis • Began Methotrexate 12.5mg weekly, Folic Acid • Close weekly to monthly follow-ups • Now increased to MTX 15mg weekly

  14. Follow up • Patient doing excellent! • ABR completely normal (6/2012) • No Flares • Labs stable

  15. Uncommon immune-mediated chronic disorder of the cartilage, thought to have autoimmune etiology Characterized by recurrent episodes of inflammation of the cartilageCan involve *ears, nose, joints, spine, eyes, tracheaEyes, heart, and blood vessels which have a similar biochemical makeup to cartilage may be affected1/3 of cases occur in association with another disease Relapsing Polychondritis

  16. Relapsing PolychondritisOther manifestations….

  17. Closing points • Chronic otitis externa unresponsive to conventional therapies deserves further workup • If external ear inflammation spares the lobule, consider diagnosis of relapsing polychondritis • SNHL requires further workup and treatment • Evaluate for further high risk associated manifestations

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