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1/5. Surgical results of cochlear implantation in malformed cochlea.

    OBJECTIVE: To report the surgical aspects of cochlear implantation in malformed cochlea. SETTING: Tertiary care center. STUDY DESIGN: Retrospective case review. methods: Between November 1997 and October 2004, 20 patients with inner ear malformations were implanted in our department. The age range was between 2 and 37 years (average, 8.8 yr). The anomalies were classified according to Sennaroglu and Saatci classification. There were two patients with common cavity deformity, four cases of incomplete partition (IP) type I (cystic cochleovestibular malformation), four cases of IP type II (classical Mondini's deformity), nine patients with large vestibular aqueduct (LVA) syndrome, and one patient with X-linked deafness. RESULTS: Standard transmastoid facial recess approach was used in 17 patients (three patients with IP I, four patients with IP II, and nine patients with LVA syndrome). In the remaining patient with IP I, because of the dehiscent and anteriorly located facial nerve, the surgical approach had to be modified, and an anteroposterior approach was used. After elevating the tympanomeatal flap, the electrode was inserted through the ear canal and then transferred to the mastoid through a full-length cut produced in the ear canal. The flap then returned to its place. In the patients with common cavity deformity, the electrode was inserted by the transmastoid labyrinthotomy approach. facial nerve had an abnormal course in four patients, but no patient had facial weakness postoperatively. Cerebrospinal fluid gusher was encountered in four patients, whereas oozing was present in five patients. It seems that a slightly larger cochleostomy may reduce postoperative rhinorrhea. The patient with common cavity deformity showed abnormal vestibular stimulation which decreased and was totally abolished during a 3-month period. CONCLUSION: Based on these findings, cochlear implantation is surgically feasible in patients with common cavity, IP types I and II, and LVA. The surgeon should be ready to make modifications in the surgical approach because of the abnormal course of the facial nerve and be ready to produce special precautions to cerebrospinal fluid gusher.
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ranking = 1
keywords = aqueduct
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2/5. Repair of a cerebrospinal fluid perilymph fistula primarily through the middle ear and secondarily by occluding the cochlear aqueduct.

    A 35-year-old man had a 5-year history of fluctuating hearing loss in his only hearing ear. history and diagnostic tests indicated a perilymph fistula, a diagnosis subsequently confirmed by exploration. Primary and secondary repairs temporarily ameliorated symptoms. A spinal fluid to middle ear fluid pathway was identified by radioactive tracer. A patent cochlear aqueduct indicated on computed tomography scan was found and repaired through a posterior cranial fossa approach. Hearing was preserved, remaining relatively stable during the 2-year follow-up period.
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ranking = 5488.2873299966
keywords = cochlear aqueduct, aqueduct
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3/5. Congenital cerebrospinal otorrhea.

    Four locations for congenital cerebrospinal fluid fistula in the region of a normal labyrinth are reviewed. A congenital leak may occur through the petromastoid canal, a wide cochlear aqueduct, Hyrtl's fissure, or the facial canal. A fistula through the initial segment of the fallopian canal was successfully repaired in a two-year-old boy who had three episodes of meningitis following otitis media. knowledge of these four sites of congenital defects provides a guideline for the surgeon in the identification and repair of cerebrospinal fluid leaks in the region of the labyrinth.
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ranking = 1097.6574659993
keywords = cochlear aqueduct, aqueduct
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4/5. Congenital deafness and spontaneous CSF otorrhea.

    Two patients with congenital deafness had spontaneous CSF otorrhea and recurrent bacterial meningitis. A careful workup, including tomograms, is especially important in the treatment of the closed type of CSF otorrhea. At operation of our patients, the leak was found to be at or near the oval window. The stapes was removed, and muscle was packed into the defect. The profuse flow of CSF was believed to be secondary to a wide-open cochlear aqueduct in the first case, and to an abnormal communication between the internal auditory meatus and the vestibule in the second case. These findings were the bases for the recurrent bacterial meningitis.
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ranking = 1097.6574659993
keywords = cochlear aqueduct, aqueduct
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5/5. Spontaneous cerebrospinal fluid otorrhea in association with a congenital defect of the cochlear aqueduct and Mondini dysplasia.

    Unrecognized spontaneous cerebrospinal fluid (CSF) otorrhea led to recurrent bacterial meningitis in three children. The underlying cause of the spontaneous CSF otorrhea was proved to be a congenital cystic dilatation of the cochlear aqueduct and Mondini dysplasia of the temporal bone. The CSF leak recurred in all patients after an initial surgical attempts to close the defects through a tympanotomy. A suboccipital approach was used successfully to obliterate the CSF fistulas in two children; a translabyrinthine approach was used in the other. A search of the literature revealed that Mondini dysplasia is a congenital anomaly that can commonly cause spontaneous CSF otorrhea in children. The authors discuss the management of this uncommon entity on the basis of a literature review and their own experience.
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ranking = 5488.2873299966
keywords = cochlear aqueduct, aqueduct
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