Otalgia, Hearing Loss, and Mastoiditis Refractory to Antibiotic Therapy

To the Editor:

A previously healthy 35-year-old woman was admitted to the johns hopkins hospital with 6 weeks of bilateral ear pain and hearing loss.

She was initially diagnosed by her primary provider with otitis media and treated with amoxicillin, without improvement. Referral to otolaryngology revealed otitis media with right tympanic membrane rupture. She underwent myringotomy, evacuation of purulent fluid (culture negative), and ciprofloxacin treatment. Due to lack of improvement, computed tomography (CT) was performed, showing right middle ear fluid and mastoid opacification. Bilateral tympanostomy tubes were placed; a third course of antimicrobial therapy was prescribed.

Notwithstanding, her symptoms worsened. She developed near complete right-sided hearing loss and difficulty detecting left-sided high-pitched sounds. Consequently, she was hospitalized for empiric intravenous antibiotic therapy. Absent were constitutional symptoms, sinus pressure, epistaxis, eye redness, rash, arthralgias, hemoptysis, or paresthesia.

On examination, she was afebrile. The right tympanic membrane was erythematous with discharge obscuring the tympanostomy tube, with right mastoid tenderness. There was mucoid discharge in the left external auditory canal. Bone conduction exceeded air conduction on the right (Rinne test). Pulmonary, cardiac and abdominal examinations were unremarkable. There was no rash, synovitis, or sensorimotor deficit.

Laboratory results were notable for erythrocyte sedimentation rate of 71 mm/h, C-reactive protein 8 mg/dL, white blood count 10.7 K/cu³, hemoglobin 10.2 g/dL, platelets 482 K/mm³, with serum creatinine 0.5 mg/dL. Urine protein-to-creatinine ratio was 0.1; urinalysis was without proteinuria or hematuria.

Chest radiograph was normal. Repeat CT imaging showed complete opacification of the right middle ear, a large right mastoid effusion, partial opacification of the left middle ear, and a moderate left mastoid effusion with clear paranasal sinuses (Figure A). Middle ear drainage yielded negative bacterial, fungal, and mycobacterial cultures. Antineutrophil cytoplasmic antibody was positive (C-ANCA 1:320). Antiproteinase 3 antibody was positive at 343 units (normal <20); antimyeloperoxidase antibody was 4 units (normal <20).

Figure.

(A) Computed tomography of the head. Red arrows point to mastoiditis (opacification of mastoid air cells) and red star identifies clear paranasal sinuses. (B) Histopathology (hematoxylin-and-eosin staining) from right mastoid biopsy with giant cells (red arrows). (C) Histopathology (hematoxylin-and-eosin staining) from right mastoid biopsy of necrobiotic debris (red arrows).

To confirm a suspected diagnosis of granulomatosis with polyangiitis (GPA), and to exclude an active mycobacterial infection, tissue biopsy was pursued. Given the absence of sinus, lung, or kidney involvement, biopsy of the right ear and mastoid bones identified dense acute and chronic inflammation with multinucleated giant cells (Figure B) and necrobiosis (Figure C). There was no evidence of vascular inflammation or granuloma formation. Acid-fast bacilli (AFB) and fungal stains and cultures were negative. These histologic features were supportive of GPA.

In an attempt to reverse hearing loss and arrest disease progression, methylprednisolone at 1000 mg intravenously daily for 3 consecutive days was initiated, followed by oral daily prednisone at 1 mg/kg/d. She reported noticeable improvement in hearing and marked reduction in ear pain within 48 hours of starting glucocorticoids. Methotrexate was commenced.

GPA is a systemic necrotizing vasculitis involving small and medium-sized vessels, predominantly affecting the upper and lower respiratory tract and kidneys. In uncommon fashion, our patient manifested isolated otalgia, hearing loss, and mastoiditis without constitutional symptoms, sinus, lung, or renal involvement.

GPA should be included in the differential diagnosis of adults presenting to the internist with recurrent otitis media refractory to antibiotic therapy or with hearing loss. Although otitis media is common in pediatric populations, it is uncommon in adults. Although otolaryngologic involvement is common with multisystem GPA, it is rarely the sole disease feature. Among 89 patients with new onset GPA, otologic involvement was observed in 35% of the cohort; 23% had otitis media/otomastoiditis, 19% had conductive hearing loss, and 12% had asymmetrical sensorineural hearing loss.¹

Some auditory recovery is possible with immunosuppressive therapy.² In our patient, after 3 weeks of therapy, speech reception threshold was 60 dB in the right ear and 30 dB in the left ear.