Giant Temporal Lobe Abscess in a Patient With Ectodermal Dysplasia

OBJECTIVE: To present the first reported case of a temporal lobe abscess in a patient with ectodermal dysplasia (ED). To increase awareness among neurotologists of the otological manifestations and complications of ED, we describe a conservative approach in comparison with the surgical strategies to repair bony defects of the tegmen tympani. STUDY DESIGN: Clinical capsule report. PATIENT: A 44-year-old man with ED presented with retroauricular pain and high-grade fever after Eustachian tube catheterizations to treat an effusive otitis media. Since many signs indicated meningitis, a computed tomography (CT) scan of the brain was performed, and an extensive intraparenchymal mass was detected. Magnetic Resonance Images (MRI) confirmed a brain abscess of the right temporal lobe. INTERVENTIONS: Surgical drainage of the abscess through craniotomy, antimicrobial therapy, and strict follow-up. MAIN OUTCOME MEASURES: Clinical symptoms and radiological signs. RESULTS: After craniotomy, the antimicrobial therapy was administered for 8 weeks. Postoperative high resolution TC of the petrous bone showed the presence of material with a soft tissue signal in the right middle ear and dehiscent anterior tegmen tympani. The location of the bony defect in association with the good clinical outcome with medical therapy mitigated against a first choice otosurgical approach to repair the dehiscence. Serial MRI scans provided proof of complete remission and no relapse occurred during a 2-year follow-up. CONCLUSIONS: Patients with ED can present with chronic otitis media and tegmen tympani defects, predisposing them to serious complications. Imaging is mandatory, and the optimum treatment requires assessment of various factors.