communication between subarachnoid and per lymphatic spaces can be due to a deficiency of lamina crib rosa (stapes gusher). recognition of the condition may alter the course of treatment that can avoid perilymph gushing. a five-year-old boy presented with a history of congenital hearing loss and recurrent meningitis. the computed tomography (ct) of the temporal bone showed severe bilateral dysp...

Background: Pseudo-Pendred syndrome (PPS) is a genetic disorder inherited as an autosomal recessive disorder. PPS characterized by the presence of hypothyroid goiter combined with mild to moderate form SNHL, not presentable at birth, no obvious inner ear malformation. This major difference from Pendred in which SNHL congenital, severe profound nature, and associated malformation, mostly enlarge...

introduction: congenital anomalies of the cochlea and labyrinth can be associated with meningitis and varying degrees of hearing loss or deafness. despite antibiotics, meningitis remains a life threatening complication.   case report: we report a case of recurrent meningitis following episodes of otitis media in a cochlear implantee child with bilateral vestibulocochlear malformation, due to fi...

esis of the inner ear. The malformation, commonly associated with deafness, is frequently heralded by meningitis secondary to communication of the middle ear with the subarachnoid space. Early recognition and successful repair of the cerebrospinal fluid leak is important to avoid the sequelae or repeated episodes of meningitis. We report a case of a 5-year-old girl in whom Mondini dysplasia pre...