Bilateral narrow duplicated internal auditory canal (original) (raw)
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Narrow duplicated internal auditory canal: radiological findings and review of the literature
Pediatric Radiology, 2005
Narrow duplicated internal auditory canal (IAC) is a rare malformation of the temporal bone that is associated with ipsilateral congenital sensorineural hearing loss. This may be an isolated finding or a part of a syndrome. Radiological examination should demonstrate aplasia or hypoplasia of the neural components of the narrow IAC, to guide the surgical approach. We report a 7-year-old boy with Klippel-Feil syndrome with a narrow double IAC with no sensorineural hearing loss but with conductive hearing loss. In this patient, the IAC consisted of two separate narrow bony canals clearly seen on 3D temporal bone CT and one nerve that was delineated on MRI. The contralateral external auditory canal was stenotic and the ossicles were dysplastic.
Bilateral duplication of the internal auditory canal
Pediatric Radiology, 2007
Duplication of the internal auditory canal is an extremely rare temporal bone anomaly that is believed to result from aplasia or hypoplasia of the vestibulocochlear nerve. We report bilateral duplication of the internal auditory canal in a 28-month-old boy with developmental delay and sensorineural hearing loss.
IIUM Medical Journal Malaysia
Duplication of the internal auditory canal is a rare entity. It can be unilateral or bilateral involvement and usually found during an investigation of sensorineural hearing loss or facial nerve palsy. We report this image finding in a child with Down syndrome with bilateral profound sensorineural hearing loss. There was no facial nerve palsy. We highlight the rarity of this image finding and the importance of imaging for detection of middle and inner ear abnormality in a case of congenital sensorineural hearing loss as it helps in decision making for the suitable treatment. Keywords: Duplicate IAC; Bilateral Duplicate IAC; sensorineural hearing loss; Down syndrome
Bilateral narrow duplication of the internal auditory canal
The Journal of Laryngology & Otology, 2010
Aim:To demonstrate diagnostic imaging of an extremely rare presentation of bilateral narrow duplication of the internal auditory canal.Case report:An adolescent boy with profound sensorineural hearing loss presented for hearing rehabilitation. Imaging studies (i.e. multidetector computed tomography and magnetic resonance imaging) clearly demonstrated bilateral duplication of the internal auditory canals, with narrowing of the lower canals, unilateral cochlear and vestibular dysplasia, bilateral superior semicircular canal malformation, and bilateral absence of the posterior semicircular canals.Conclusion:To our knowledge, this is only the third such case described in the literature. Considering that the vestibulocochlear nerve has been unable to be demonstrated in almost all cases of duplicated internal auditory canal (unilateral and bilateral), our case supports the hypothesis that vestibulocochlear nerve aplasia or hypoplasia leads to internal auditory canal stenosis. We consider ...
Narrow, duplicated internal auditory canal
Neuroradiology, 2003
A narrow internal auditory canal (IAC) constitutes a relative contraindication to cochlear implantation because it is associated with aplasia or hypoplasia of the vestibulocochlear nerve or its cochlear branch. We report an unusual case of a narrow, duplicated IAC, divided by a bony septum into a superior relatively large portion and an inferior stenotic portion, in which we could identify only the facial nerve. This case adds support to the association between a narrow IAC and aplasia or hypoplasia of the vestibulocochlear nerve. The normal facial nerve argues against the hypothesis that the narrow IAC is the result of a primary bony defect which inhibits the growth of the vestibulocochlear nerve.
A Challenge for Cochlear Implantation: Duplicated Internal Auditory Canal
The Journal of International Advanced Otology, 2016
Duplication of the internal auditory canal is an uncommon, congenital malformation that can be associated with sensorineural hearing loss owing to aplasia/hypoplasia of the vestibulocochlear nerve. Only 14 such cases have been reported to date. We report the case of a 13-month-old girl with bilateral, congenital, sensorineural hearing loss caused by narrow, duplicated internal auditory canals and discuss the challenges encountered in the diagnosis and treatment of this condition.
Vertically oriented internal auditory canal in an 8-year-old with hearing loss
International journal of pediatric otorhinolaryngology, 2006
To report a unique orientation of the internal auditory canal and possible association with congenital hearing loss. Retrospective chart review of an 8-year-old Hispanic male with a mixed hearing loss. Uniquely abnormal orientation of the internal auditory canal in a patient with apparently normal cochleovestibular structures and a mixed hearing loss. A vertically oriented IAC is a rarely described anatomical anomaly of the temporal bone that may have associations with congenital hearing loss.
Abnormal direction of internal auditory canal and vestibulocochlear nerve
The Journal of Laryngology & Otology, 2004
Several internal auditory canal (IAC) anomalies have been reported. To our knowledge, only one case with an abnormal direction of the IAC has been reported in an infant with Pierre Robin syndrome. In this paper, we present the first report of two non-syndromic cases with abnormal IAC direction.
Bilateral congenital semicircular canal malformation and hearing loss - case report
NASCER E CRESCER - BIRTH AND GROWTH MEDICAL JOURNAL, 2020
The main aims of this observational study were to describe a poorly characterized malformation of the inner ear termed bilateral congenital semicircular canal malformation; determine if the degree and pattern of semicircular canal dysmorphology and the presence or absence of a well-formed cochlea predict audiological outcomes, type, and severity of congenital hearing loss; and investigate its relationship with known syndromic forms of hearing loss. Review of eight cases of hearing loss with radiographic evidence of congenital semicircular canal malformation was performed. Information was collected on clinical history, physical examination, computed tomography study and serial audiograms for all patients. Analyzed features included other syndrome-characteristic phenotypic dysmorphologies, audiometric configuration, severity and type of hearing loss, type of audiological rehabilitation, and presence of associated inner ear abnormalities besides those in the vestibular system. Among th...
CT and MR imaging of congential abnormalities of the inner ear and internal auditory canal
European Journal of Radiology, 2001
The embryology of the inner ear must be known as many of the inner ear malformations present as a result of the arrest during the various stages of embryology. These malformations are described in this 'embryologic' perspective and specific names for certain malformations are no longer used. Both CT and MR can be used to look at inner ear malformations but often both techniques are complementary. However, CT is preferred when associated middle-or external ear malformations must be excluded. Magnetic resonance is preferred when subtle changes in the membranous labyrinth or abnormalities of the nerves in the internal auditory canal must be visualised. The CT and MR technique must however be adapted as more and more subtle congenital malformations can only be seen when the right technique is used. The heavily T2-weighted gradient-echo or fast spin-echo MR techniques are mandatory if malformations of the inner ear must be excluded. The purpose of this paper is to describe the techniques used to study these patients and to give an overview of the most frequent and important congenital malformations which can be found in the inner ear and internal auditory canal/cerebellopontine angle. : S 0 7 2 0 -0 4 8 X ( 0 1 ) 0 0 3 7 7 -1