RESUMO
OBJECTIVE: To perform an otopathologic analysis of temporal bones (TBs) with CHARGE syndrome. STUDY DESIGN: Otopathologic study of human TB specimens. SETTING: Otopathology laboratories. METHODS: From the otopathology laboratories at the University of Minnesota and Massachusetts Eye and Ear Infirmary, we selected TBs from donors with CHARGE syndrome. These TBs were serially sectioned at a thickness of 20 µm, and every 10th section was stained with hematoxylin and eosin. We performed otopathologic analyses of the external ear, middle ear (middle ear cleft, mucosal lining, ossicles, mastoid, and facial nerve), and inner ear (cochlea, vestibule, internal auditory canal, and cochlear and vestibular nerves). The gathered data were statistically analyzed. RESULTS: Our study included 12 TBs from 6 donors. We found a high prevalence of abnormalities affecting the ears. The most frequent findings were stapes malformation (100%), aberrant course of the facial nerve (100%) with narrow facial recess (50%), sclerotic and hypodeveloped mastoids (50%), cochlear (100%) and vestibular (83.3%) hypoplasia with aplasia of the semicircular canals, hypoplasia and aplasia of the cochlear (66.6%) and vestibular (91.6%) nerves, and narrowing of the bony canal of the cochlear nerve (66.6%). The number of spiral ganglion and Scarpa's ganglion neurons were decreased in all specimens (versus normative data). CONCLUSIONS: In our study, CHARGE syndrome was associated with multiple TB abnormalities that may severely affect audiovestibular function and rehabilitation.
Assuntos
Síndrome CHARGE/complicações , Orelha Interna/anormalidades , Orelha Média/anormalidades , Osso Temporal/anormalidades , Anormalidades Múltiplas , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Michigan , MinnesotaRESUMO
HYPOTHESIS: The presence of bony inner ear malformations may associate with a number of anatomical abnormalities affecting the middle ear structures. Those malformations may create pitfalls and complications for cochlear implantation. BACKGROUND: Inner ear malformations associate with varying degrees of hearing loss, and frequently require cochlear implantation for hearing rehabilitation. Therefore, the abnormalities affecting the middle- and inner-ear structures may increase the risk of surgical complications. METHODS: We examined 38 human temporal bones from donors with bony inner ear malformations. Using light microscopy, we analyzed the presence of abnormalities in the structures of the middle- and inner-ear. RESULTS: Our collection comprises of 38 specimens with inner-ear malformations (cochlear aplasia, nâ=â3; cochlear hypoplasia, nâ=â30; incomplete partition, nâ=â3; isolated vestibular malformation, nâ=â2). The anatomy of the middle ear was abnormal in most temporal bones with cochlear aplasia, cochlear hypoplasia, and incomplete partition type I (40%-100%). Some of those abnormalities (hypoplastic or obliterated mastoid, 55.2%; aplastic or obliterated round window, 71.0%; aberrant course of the facial nerve, 36.8%) may hinder the access to the round window using the conventional facial recess approach for cochlear implantation. The cochlear nerve and associated bony structures (internal auditory canal and bony canal for cochlear nerve) were normal in 71.0% of all temporal bones with inner ear malformations. CONCLUSION: Each different type of malformation may create specific surgical challenges to surgeons. Comprehensive preoperative imaging is fundamental toward the surgical success of cochlear implants in patients with malformations. Alternatives to circumvent those middle- and inner-ear abnormalities and potential complications are further discussed.
Assuntos
Implante Coclear/métodos , Orelha Interna/anormalidades , Orelha Média/anormalidades , Implantes Cocleares/efeitos adversos , Orelha Interna/cirurgia , Orelha Média/cirurgia , Feminino , Humanos , Masculino , Tomografia Computadorizada por Raios XRESUMO
UNLABELLED: Bell's palsy is hypothesized to result from virally mediated neural edema. Ischemia occurs as the nerve swells in its bony canal, blocking neural blood supply. Because viral infection is relatively common and Bell's palsy relatively uncommon, it is reasonable to hypothesize that there are anatomic differences in facial canal (FC) that predispose the development of paralysis. Measurements of facial nerve (FN) and FC as it follows its tortuous course through the temporal bone are difficult without a 3D view. In this study, 3D reconstruction was used to compare temporal bones of patients with and without history of Bell's palsy. METHODS: Twenty-two temporal bones (HTBs) were included in the study, 12 HTBs from patients with history of Bell's palsy and 10 healthy controls. Three-dimensional models were generated from HTB histopathologic slides with reconstruction software (Amira), diameters of the FC and FN were measured at the midpoint of each segment. RESULTS: The mean diameter of the FC and FN was significantly smaller in the tympanic and mastoid segments (p = 0.01) in the BP group than in the controls. The FN to FC diameter ratio (FN/FC) was significantly bigger in the mastoid segment of BP group, when compared with the controls. When comparing the BP and control groups, the narrowest part of FC was the labyrinthine segment in control group and the tympanic segment in the BP. CONCLUSION: This study suggests an anatomic difference in the diameter of FC in the tympanic and mastoid segments but not in the labyrinthine segment in patients with Bell's palsy.