Giant Temporal Lobe Abscess in a Patient With Ectodermal Dysplasia

Area of Science:

• Neurology and Otolaryngology
• Neurotology
• Genetics and Rare Diseases

Background:

• Ectodermal dysplasia (ED) is a group of inherited disorders characterized by abnormalities in hair, teeth, nails, and sweat glands.
• Otological manifestations, including chronic otitis media and tegmen tympani defects, are recognized complications of ED.
• Brain abscesses are rare but serious complications that can arise from middle ear infections, particularly in patients with predisposing anatomical factors.

Observation:

• A 44-year-old male patient with a history of ED presented with symptoms suggestive of meningitis, including retroauricular pain and fever.
• Initial investigations revealed an extensive intraparenchymal mass on computed tomography (CT), confirmed as a right temporal lobe brain abscess by magnetic resonance imaging (MRI).
• The patient had a history of effusive otitis media treated with Eustachian tube catheterization.

Findings:

• The patient underwent surgical drainage of the abscess via craniotomy, followed by an 8-week course of antimicrobial therapy.
• Postoperative imaging identified a dehiscent anterior tegmen tympani, a bony defect in the skull base above the middle ear.
• Despite the tegmen tympani defect, conservative management with medical therapy led to complete remission of the abscess, with no relapse during a 2-year follow-up.

Implications:

• This case underscores the potential for serious neurological complications, such as brain abscesses, in patients with ectodermal dysplasia.
• The findings suggest that a conservative, non-surgical approach to tegmen tympani defects may be successful in select cases of brain abscess associated with ED, especially with good clinical response to medical therapy.
• Neurotologists and clinicians should maintain a high index of suspicion for otological and neurological complications in patients with ED and ensure thorough imaging and individualized treatment strategies.