The aim of this study is to survey and compare the development of auditory skills in young children with Mondini dysplasia and profoundly-deaf young children with radiologically normal inner ears over a period of 3 years after cochlear implantation. A total of 545 young children (age 7 to 36 months) with prelingual, severe to profound hearing loss participated in this study. All children receiv...

OBJECTIVES/HYPOTHESIS In this study, our objective was to histopathologically analyze the peripheral vestibular system in patients with Mondini dysplasia. STUDY DESIGN Comparative human temporal bone study. METHODS We assessed the sensory epithelium of the human vestibular system with a focus on the number of type I and type II hair cells, as well as the total number of hair cells. We compa...

Mondini dysplasia, also known as Mondini malformation, is a developmental abnormality of the inner and middle ears that can cause hearing loss, cerebrospinal fluid (CSF) leakage, and recurrent bacterial meningitis (RBM), which is defined as two or more episodes of meningitis separated by a period of convalescence and the complete resolution of all signs and symptoms. An accurate diagnosis of th...

OBJECTIVE To study a family with inner ear malformations and sensorineural hearing loss. DESIGN Clinical, radiological, and genetic study of the members of a family with different degrees of sensorineural hearing loss. RESULTS The males in the family manifested profound congenital hearing loss with severe inner ear malformations, while the only affected female had progressive hearing loss t...

recurrent bacterial meningitis is not a common disease and makes physicians seek underlying predisposing factors which can result from anatomic anomalies or immunodeficiency. in this paper we present a boy with recurrent bacterial meningitis with the history of trauma and sensorineural hearing loss. mondini dysplasia was demonstrated with computed homographic scans (ct-scan) of temporal bones.

Klippel-Feil syndrome is defined as the fusion of cervical vertebra with associated congenital anomalies but was rarely reported to be associated with Mondini Malformation. We report a newborn girl with severe neck extension, computed tomography (CT) of the neck after birth showed fusion of the fifth, sixth, and seventh cervical vertebrae, compatible with Klippel-Feil Syndrome and CT temporal b...

In 1791 , Mondini described a membranous and bony dysplasia of the inner ear in a congenitally deaf child (1]. Classically, the Mondini deformity consists of a cochlea containing a normal basal turn and a total of 1.5 rather than 2.5 turns; the middle and apical turns are incorporated into a single sac. Because dysplasia of the labyrinth may be associated with dural dehiscence and a bony defect...

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...