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Barakat syndrome is a rare autosomal dominant disease caused by haplo-insufficiency of transcription factor GATA3 on chromosome 10p14. It is characterized by a triad of hypoparathyroidism, sensorineural hearing loss, and renal disease with high phenotypic heterogeneity. Here we report a case of Barakat syndrome in a newborn presenting with isolated sensorineural hearing loss. A comprehensive set of diagnostic tests, including blood work and imaging were unremarkable outside of the failed newborn hearing screening. Recognizing Barakat syndrome is clinically challenging given the highly variable presentation. Otolaryngologists should consider Barakat syndrome when evaluating patients presenting with isolated congenital sensorineural hearing loss.

Nontuberculous mycobacteria (NTM) are ubiquitous in nature and most commonly cause pulmonary infections and cervicofacial lymphadenitis in children. Here we report a rare case of NTM mastoiditis in a 3-year-old immunocompetent patient who presented with left mucoid otorrhea refractory to two months of ciprofloxacin/dexamethasone drops. The patient underwent left cortical mastoidectomy and treatment with 7 months of triple antibiotic therapy. Infection remained localized to the middle ear with signs of resolution. Early bacterial culture with acid-fast bacilli staining should be considered in immunocompetent patients presenting with refractory otorrhea.