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...

performing cochlear implantation in patients with inner ear malformation has always been a matter of dispute. this study was designed to analyze the operative findings,complications, and postoperative performance of patients with inner ear anomalies who underwent cochlear implantation. six patients with inner ear malformations underwent implantation in our academic tertiary referral center from...

In the ENT region 50% of the malformations affect the ear. Malformations of the outer and middle ear are predominantly unilateral (ca. 70-90%) and mostly involve the right ear. Inner ear malformations can be unilateral or bilateral. The incidence of ear malformations is approximately 1 in 3800 newborns. Ear malformations may be genetic (associated with syndromes or not, with family history, spo...

Malformations of bony inner ear are rare anomalies occurring in approximately 20% of patients with congenital sensorineural hearing loss. Conductive hearing loss is usually associated with abnormalities of the external and middle ear. Recent reports of patients with lateral semicircular canal malformations indicate inner ear malformations to be associated with sensorineural or conductive hearin...

OBJECTIVE To explore the clinical experience of multi-channel cochlear implantation in patients with bilateral inner ear malformations. METHOD Multi-channel cochlear implantation was conducted among 180 patients from 1997 to 2001. Twenty-five of them, being with inner ear malformations, were given hearing and speech rehabilitation including sound detection, word discrimination, auditory compr...

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...

Spontaneous cerebrospinal fluid (CSF) fistula in congenital malformations of the inner ear is rare, and it is found most commonly in infants and children with impaired hearing. Spontaneous CSF otorrhea in adults is often due to defects in the tegmen and the bone overlying the posterior fossa in an otherwise normal ear. In the literature, there are few reports of adultonset spontaneous CSF otorr...

OBJECTIVE/HYPOTHESIS To assess the audiologic and surgical outcomes for pediatric cochlear implant patients with inner ear malformations. STUDY DESIGN Retrospective review of 315 pediatric cochlear implant cases from 1994 to 2002. METHODS Twenty-eight pediatric cochlear implant patients with known inner ear malformations determined on high-resolution computed tomography (HRCT) of the tempor...

OBJECTIVES/HYPOTHESIS To assess the hearing status of trisomy 21 patients by analyzing electrophysiological and radiological findings of any correlation between hearing impairment and major or minor inner ear malformations. STUDY DESIGN Prospective radiological and electrophysiological study. METHODS A group of 34 ears of Down syndrome subjects and 20 ears of a volunteer age- and sex-matche...

PURPOSE To determine CT findings in the external, middle, and inner ear of patients with microtia and external auditory canal dysplasia. METHODS We used high-resolution CT, with multiplanar or axial 1-mm continuous sections, coronal or sagittal reformations, or low-dose spiral acquisitions, to examine 184 temporal bones of children with microtia. RESULTS In cases of minor microtia, auditory...