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Gonçalo Almeida1, Bruno Cunha1, Marta Conde2, Herédio Sousa3, Rui Carvalho1, Carla Conceição1

1 – Serviço de Neurorradiologia, Hospital Dona Estefânica e Hospital são José, CHULC
2 – Unidade de Reumatologia Pediátrica, Área da Mulher, Criança e Adolescente, Hospital Dona Estefânica, CHULC
3 – Serviço de Otorrinolaringologia, Hospital Dona Estefânica, CHULC

- XV Congresso Nacional da SPNR, 28-29 Junho 2019, Coimbra
- Neuroradiology (2020) 62:109–127.

Introduction: Chronic inflammatory diseases of the ear include a range of conditions involving the external, middle and inner ear, requiring medical and/or surgical treatment. Complications include cholesteatoma with destructive changes, meningitis, hearing loss, labyrinthitis and other conditions, with frequent hospitalization.
Material and Methods: We present two cases of two year-old children presenting with persistent otorrhea in the context of chronic inflammatory diseases of the ear.
Results: In both cases CT and MRI imaging showed ossicular erosion and soft tissue opacification of the external and middle ears (bilaterally in one case) with heterogeneous signal intensity on T2WI and enhancement after gadolinium injection. They also had unilateral destructive changes of the inner ear, labyrinthitis and enhancement within the internal auditory canal. Both patients had multiple hospital admissions with increased otorrhea and resistance to antibiotic therapy. Several biopsies were inconclusive except two, showing foreign body granulomas in one case and evidence of vasculitis in the other. Tuberculosis, Langerhans cell histiocytosis, sarcoidosis, granulomatosis with polyangiitis and primary immunodeficiency were excluded. In one case a subsequent MRI showed an area of restricted diffusion adjacent to the external auditory canal and an epitympanic lesion with no suppression in the T1FS sequence. The histological study confirmed the presence of a left ear cholesteatoma and cholesterol granuloma. In the other case the patient presented months later with fever and contralateral sensorineural hearing loss. An MRI revealed pachymeningeal, leptomeningeal and contralateral VIII cranial nerve enhancement, with laboratorial evidence of aseptic meningitis. Both patients also developed labyrinthitis ossificans in subsequent imaging studies. Clinical improvement was achieved after the introduction of immunosuppressive medication, without further progression of the destructive process.
Conclusion: Chronic inflammatory diseases of the ear represent a diagnostic challenge and remain frequently misunderstood. Its differential diagnosis must be taken into account in order to provide better treatments to patients.

Palavras Chave: Chronic inflammatory disease; ear; granuloma; destructive changes; imaging