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

2. 11y/o female presents to ENT clinic….  Several years of otalgia associated 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. 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 conchal bowl. HT – Bilateral mixed mod-severe HL, nl tymps RX - 1/2str vinegar, external ear moisturization changed to steroid cream/moisturization & dermotic CT scan temporal bones ordered

5. 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.  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.  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.  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.  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.  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

12.  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. Relapsing Polychondritis  Began Methotrexate 12.5mg weekly, Folic Acid  Close weekly to monthly follow-ups  Now increased to MTX 15mg weekly

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

15. Relapsing Polychondritis

16. 

17.  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