Spontaneous cerebrospinal fluid otorrhea in a deaf infant

Spontaneous (congenital) cerebrospinal fluid (CSF) otorrhea is a rare occurrence with less than 100 cases being reported in the literature. In infants and children, it may present as otitis media with effusion. A 4-month-old infant, who was diagnosed with severe to profound sensorineural hearing impairment and spontaneous CSF otorrhea, initially presented with otitis media with effusion. The patient presentation, diagnostic dilemmas and pathophysiology of this rare but interesting problem are discussed.