摘要:

AbstractApproximately 20% of patients with congenital sensorineural hearing loss have radiographic abnormalities of the inner ear. A broad spectrum of anomalous patterns have been described, most of which have been lumped together under the term “Mondini's dysplasia.” We feel that this grouping of many dissimilar entities under a single umbrella term is unwarranted. Based on a review of 63 patients with 98 congenitally malformed ears, we have been able to recognize a number of distinct anatomic patterns from their radiographic appearance. A remarkable similarity between these morphologies and the appearance of the inner ear at various stages of embryogenesis was found. This led us to propose a classification system based upon the theory that these deformities result from an arrest of development during varying stages of inner ear organogene

ISSN:0023-852X    

DOI:10.1002/lary.5540971301

出版商:John Wiley&Sons, Inc.    

年代:1987

数据来源: WILEY

摘要:

AbstractTo determine if the cochlear implant can enable sound detection in children with a congenitally deformed cochlea, we reviewed warble‐tone thresholds in five ears of four children in the implant clinical trials program. Of the five ears, there were two common cavity deformities, two cochlear hypoplasias, and one incomplete partition. Four of the five ears had an auditory response to stimulation by the implant at the same level as ears deafened by other disorders. One ear that had an auditory response to stimulation also produced facial stimulation that precluded use of the implant. The one ear that did not stimulate was the incomplete partition. This ear had a very narrow internal auditory canal. Three patients are now using the implant in three ears to detect sound.Results show that a cochlear implant may enable sound detection in a patient with a malformed cochlea but that a very narrow internal auditory canal (less than 1.5 mm) detected preoperatively on radiographs may contraindicate an implant. Such anatomy suggests only a rudimentary audiovestibular nerve or no such nerve and only a facial nerv

ISSN:0023-852X    

DOI:10.1002/lary.5540971302

出版商:John Wiley&Sons, Inc.    

年代:1987

数据来源: WILEY

摘要:

AbstractThe histopathologic findings in five temporal bones from three patients with congenital malformation of the inner ear are described. The external contour of the cochlea was deformed in two temporal bones, while the internal architecture was abnormal in all five temporal bones. Intracochlear abnormalities included defects in the interscalar septum, deficiencies in the modiolus, and a paucity of neural elements, Spiral ganglion cell populations varied, but were substantially diminished in all five temporal bones. Enlargement of the vestibule and semicircular canals (lateral and posterior) was seen in two temporal bones. A large vestibular aqueduct and saccular hydrops were found in one temporal bone each.An attempt was made to relate the histologic patterns of deformity to the proposed clinical classification of inner ear malformations. Radiographic abnormality of the cochlea would have been detectable in two bones (incomplete partition pattern), while two other bones would have appeared as vestibule‐semicircular canal syndromes. One bone would have been radiographically norma

ISSN:0023-852X    

DOI:10.1002/lary.5540971303

出版商:John Wiley&Sons, Inc.    

年代:1987

数据来源: WILEY