Temporal bone histopathological findings in campomelic dysplasia

1992 ◽  
Vol 106 (4) ◽  
pp. 361-365 ◽  
Author(s):  
H. Takahashi ◽  
I. Sando ◽  
H. Masutani

AbstractBoth temporal bones of a newborn (35 gestational weeks old) with campomelic syndrome were studied histopathologically. This is to our knowledge the second temporal bone report (third case) of this syndrome. The findings included: abnormal cartilagenous and osseous tissues and abnormality in the globuli interossei in the otic capsule; deformities of the vestibule and semicircular canals, probably due to compression by the abnormal cartilaginous tissue; hypoplastic cochleaand semicircular canals; aberrant course of the facial nerve; wide dehiscence of the facial canal in the tympanic portion; slight hypoplasia of the malleus and anomalies in the incus and stapes; and large epitympanic space. These findings closely resembled those of the first report, and suggest that: 1) campomelic dysplasia is a definite disease entity with consistent pathogenesis, and 2) similar otologic manifestations may be expected in the majority of patients with this syndrome.

2008 ◽  
Vol 139 (2_suppl) ◽  
pp. P190-P190
Author(s):  
Alaa A. Abou-Bieh ◽  
Thomas J Haberkamp ◽  
Jarah Ali Al-Tubaikh

Problem The gross anatomical variations of the stapedius muscle and its relations to the facial nerve canal. Methods Thirty-five temporal bones were dissected, and the anatomic details were studied utilizing an operating microscope and otoendoscopes with 0o, 30o and 70o angles and 2.7 and 3 mm diameters. The muscle origin, its course in its bony sulcus with its relation to the facial nerve canal, the tendon and its insertion were studied. Results Marked variations in the origin, size, and course of the muscle in its bony sulcus were detected. The shape of the sulcus itself and its relation to the facial nerve canal varied also, both mainly influenced by the sinus tympani development. These variations affected the shape and length of the tendon and the pyramidal eminence. In addition, they influenced the site of tendon insertion into the stapes. The presence of ectopic muscle bundles was confirmed in one specimen. Conclusion The stapedius muscle anatomy can vary significantly from one temporal bone to another. In some situations these variations can be of surgical importance worse enough to be recognized. Significance To add important unrecognized data to the surgical anatomy of the temporal bone.


1986 ◽  
Vol 100 (7) ◽  
pp. 749-758 ◽  
Author(s):  
D. W. Proops ◽  
W. M. Hawke ◽  
G. Berger

AbstractMicrofractures of the temporal bone have been recognized for more than seventy years and occur in certain well-defined areas of the otic capsule. The etiology and significance of these microfractures has been debated since their discovery. The most commonly held theory is that they result from stresses within the otic capsule that develop with growth. An alternative explanation might be that they result from masticatory stress placed upon the temporal bone.Fifty serially-sectioned temporal bones from the Ontario Temporal Bone Bank were examined in this study for the presence of microfractures. Our findings as to the most common sites of microfractures agree with previous studies.It is proposed that the constant stress placed upon the petrous temporal bone by the act of mastication is directed by the anatomy of the external, middle and inner ear through certain definite pathways, including the Fallopian canal, the bony semicircular canals and the cochlea. The most common sites of these fractures represent sites of weakness in the path of these directed forces within the otic capsule, and the statistically significant increase in the number of fractures with age lends support to the masticatory stress theory.


1986 ◽  
Vol 27 (6) ◽  
pp. 637-644 ◽  
Author(s):  
H. Wilbrand ◽  
W. Rauschning ◽  
G. Ruhn

The subarcuate channel conveys the blood supply to the otic capsule of the semicircular canals, to part of the vestibule and to other neighbouring structures. It is often observed in conventional radiographs of the temporal bone and is reproduced to advantage by conventional multidirectional and computed tomography. The purpose of this report is to draw the attention of otoradiologists to this anatomic structure and to report on some results concerning the radioanatomy of the channel. Plastic casts of 100 radiographed and subsequently macerated temporal bones were evaluated. Fourteen temporal bone specimens were submitted to automatic serial cryomicrotomy. The radioanatomic variations of the channel were demonstrated The radiographic reproducibility of the channel, length and width, were found to be dependent on the degree of perilabyrinthine and mastoid pneumatization of the temporal bone.


2021 ◽  
pp. 019459982110089
Author(s):  
Rafael da Costa Monsanto ◽  
Renata Malimpensa Knoll ◽  
Norma de Oliveira Penido ◽  
Grace Song ◽  
Felipe Santos ◽  
...  

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.


2017 ◽  
Vol 22 (1) ◽  
pp. 41-49 ◽  
Author(s):  
Nadine Schart-Morén ◽  
Sune Larsson ◽  
Helge Rask-Andersen ◽  
Hao Li

Objective: The aim was to study the relationship between the labyrinthine portion (LP) of the facial canal and the cochlea in human inner ear molds and temporal bones using micro-CT and 3D rendering. A reduced cochlea-facial distance may spread electric currents from the cochlear implant to the LP and cause facial nerve stimulation. Influencing factors may be the topographic anatomy and otic capsule properties. Methods: An archival collection of human temporal bones underwent micro-CT and 3D reconstruction. In addition, cochlea-facial distance was assessed in silicone and polyester resin molds, and the association between the LP and upper basal turn of the cochlea was analyzed. Results: Local thinning of the otic capsule and local anatomy may explain the development of cochlea-facial dehiscence, which was found in 1.4%. A reduced cochlea-facial distance was noted in 1 bone with a superior semicircular canal dehiscence but not in bones with superior semicircular canal “blue line.” The otic capsule often impinged upon the LP and caused narrowing. Conclusion: Micro-CT with 3D rendering offers new possibilities to study the topographic anatomy of the human temporal bone. The varied shape of the cross-section of the LP could often be explained by an “intruding” cochlea.


Author(s):  
Kiran Natarajan ◽  
Koka Madhav ◽  
A. V. Saraswathi ◽  
Mohan Kameswaran

<p>Bilateral temporal bone fractures are rare; accounting for 9% to 20% of cases of temporal bone fractures. Clinical manifestations include hearing loss, facial paralysis, CSF otorhinorrhea and dizziness. This is a case report of a patient who presented with bilateral temporal bone fractures. This is a report of a 23-yr-old male who sustained bilateral temporal bone fractures and presented 18 days later with complaints of watery discharge from left ear and nose, bilateral profound hearing loss and facial weakness on the right side. Pure tone audiometry revealed bilateral profound sensori-neural hearing loss. CT temporal bones &amp; MRI scans of brain were done to assess the extent of injuries. The patient underwent left CSF otorrhea repair, as the CSF leak was active and not responding to conservative management. One week later, the patient underwent right facial nerve decompression. The patient could not afford a cochlear implant (CI) in the right ear at the same sitting, however, implantation was advised as soon as possible because of the risk of cochlear ossification. The transcochlear approach was used to seal the CSF leak from the oval and round windows on the left side. The facial nerve was decompressed on the right side. The House-Brackmann grade improved from Grade V to grade III at last follow-up. Patients with bilateral temporal bone fractures require prompt assessment and management to decrease the risk of complications such as meningitis, permanent facial paralysis or hearing loss. </p>


Author(s):  
B. Y. Praveen Kumar ◽  
K. T. Chandrashekhar ◽  
M. K. Veena Pani ◽  
Sunil K. C. ◽  
Anand Kumar S. ◽  
...  

<p class="abstract"><strong>Background:</strong> The hallmark of the temporal bone is variation. Various important structures like the facial nerve run in the temporal bone at various depths which can be injured during mastoidectomy.</p><p class="abstract"><strong>Methods:</strong> Twenty wet cadaveric temporal bones were dissected. A cortical mastoidectomy was performed followed by a canal wall down mastoidectomy and the depth of the vertical segment of the facial nerve in the mastoid was determined.  </p><p class="abstract"><strong>Results:</strong> The mean depth of the second genu was 13.82 mm. The mean depth of the stylomastoid foramen was 12.75 mm and the mean distance from the annulus at 6’0 clock to the stylomastoid foramen was 10.22 mm.</p><p><strong>Conclusions:</strong> There is significant variation in the average depth of the facial nerve in the mastoid. </p>


Author(s):  
Nathaniel Yang

Dear Editor: In the article entitled “Facial Paralysis in Longitudinal versus Oblique and Otic-Sparing versus Non Otic-Sparing Temporal Bone Fracture” published in the Vol 34 No 2 issue of the Philippine Journal of Otolaryngology Head and Neck Surgery, the authors included an image (Figure 4) that was representative of an otic-disrupting fracture.  The arrow clearly shows the fracture line running through the mastoid air cell system nearly parallel to the posterior external auditory canal wall and ending just posterior to the ossicular chain. However, the fracture line does not unequivocally appear to proceed medially towards the otic capsule, which is the densest portion of the temporal bone that surrounds the osseous labyrinth, and includes the cochlea, vestibule and semicircular canals. The otic capsule likewise is located within the petrous portion of the temporal bone.1 The otic capsule lies within the oval ring in the smaller figure above which is an enlarged image of the original figure in the article. This issue is relevant because the authors are classifying subjects according to the visual presence of a fracture that may or may not involve the otic capsule. Misidentification of the type of fracture in a representative image raises the question of a systematic misidentification in the entire data set, thus rendering the results of the study invalid.


2004 ◽  
Vol 118 (2) ◽  
pp. 106-111 ◽  
Author(s):  
Peidong Dai ◽  
Tianyu Zhang ◽  
Keqiang Wang ◽  
Jichang Song ◽  
Wen Qian ◽  
...  

The quantitative relationships between the position of the mastoid segment of the facial nerve, mastoid pneumatization and the positions of neighbouring structures are undetermined. Using high resolution computed tomography (HRCT), the positions of these structureswere measured in 66 normal temporal bones. A new method for measuring the volume of pneumatization in the temporal bone based on the serial digital images of CT was designed. The method of partial correlation analysis was used to find the real relationship of the two variables. The results suggest that the factors that influence the position of the mastoid segment of the facialnerve are complicated and multiple. The development of the cranium including the temporal bone,the temporal bone pneumatization and the variable position of the jugular bulb are considered to be important factors.


1986 ◽  
Vol 95 (5) ◽  
pp. 480-486 ◽  
Author(s):  
Charles G. Wright ◽  
William L. Meyerhoff ◽  
O. E. Brown ◽  
J. C. Rutledge

CHARGE association is a recently described cluster of congenital defects including ocular coloboma, heart disease, choanal atresia, retarded development and/or CNS abnormalities, genital hypoplasia, and ear anomalies. Although congenital hearing loss has been reported in CHARGE association, no information regarding the underlying temporal bone disease is available in the literature to date. The authors evaluated four patients with multiple anomalies consistent with CHARGE syndrome. Two surviving patients have bilateral severe hearing loss on auditory brain stem response testing. Two patients did not survive, and their temporal bones were obtained at autopsy for histologic examination. All four temporal bones showed severe middle ear defects including ossicular deformities, absence of the stapedius muscle, absence of the oval window, aberrant course of the facial nerve, and dehiscence of the facial nerve canal. In the more severely affected case, a Mondini-type malformation of the cochlea was present, together with multiple anomalies of the vestibular apparatus. Vestibular defects also occurred in the other case; however, the cochleae were found to be normally developed.


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