Acrocephalosyndactyly (Apert's Syndrome): Temporal Bone Findings

1975 ◽  
Vol 84 (2) ◽  
pp. 174-178 ◽  
Author(s):  
John R. Lindsay ◽  
Franklin O. Black ◽  
William H. Donnelly
Keyword(s):  
Cerebrospinal Fluid ◽  
Hearing Impairment ◽  
Temporal Bone ◽  
Histologic Examination ◽  
Cochlear Aqueduct ◽  
Stapes Footplate ◽  
Apert's Syndrome ◽  
Conductive Hearing

The otologic manifestation of acrocephalosyndactyly (Apert's syndrome) has usually consisted of bilateral conductive hearing impairment. Invariably, fixation of the stapes footplate has been found at tympanotomy. Manipulation or removal of the stapes has resulted in a copious flow of fluid from the vestibule, suggesting an unusually patent cochlear aqueduct with escape of cerebrospinal fluid. Histologic examination of a temporal bone from an infant with acrocephalosyndactyly showed cartilaginous fixation of the stapes footplate. The lumen of the cochlear aqueduct was not unusually large or widely patent in this case.

scholarly journals Transmastoid Repair of Temporal Meningoencephaloceles and Cerebrospinal Fluid Otorrhea

2011 ◽  
Vol 3 (1) ◽  
pp. 31-41 ◽  
Author(s):  
Werner Garavello ◽  
Lorenzo Gaini ◽  
Diego Zanetti
Keyword(s):  
Cerebrospinal Fluid ◽  
Temporal Bone ◽  
Process Management ◽  
Diagnostic Process ◽  
Mastoid Cavity ◽  
Tegmen Tympani ◽  
Surgical Options ◽  
Conductive Hearing ◽  
Transmastoid Approach ◽  
Csf Leaks

ABSTRACT This paper reviews the diagnosis and treatment of temporal bone meningoencephaloceles, defined as the herniation of meninges or brain tissue into empty spaces within the temporal bone, i.e. tympanic or mastoid cavity, through the tegmen tympani or antri respectively. It also describes the current methods of control of cerebrospinal fluid (CSF) leaks, which commonly present as serous otorrhea or rhinorrhea in addition to a variety of symptoms, such as conductive hearing loss. Imaging is the mainstay of the diagnostic process. Management of the condition is surgical, and this review outlines the surgical options with special emphasis on the transmastoid approach and the materials applicable for repair of the bony dehiscences.

Klippel-Feil Syndrome with Conductive Deafness and Histological Findings of Removed Stapes

1983 ◽  
Vol 92 (2) ◽  
pp. 202-206 ◽  
Author(s):  
Makoto Sakai ◽  
Atsushi Shinkawa ◽  
Hirosato Miyake ◽  
Nobuyuki Komatsu
Keyword(s):  
Hearing Impairment ◽  
Cervical Vertebrae ◽  
Sudden Hearing Loss ◽  
Oval Window ◽  
Conductive Deafness ◽  
Sensorineural Hearing Impairment ◽  
Stapes Footplate ◽  
Osseous Changes ◽  
The Right ◽  
Conductive Hearing

The Klippel-Feil syndrome is usually associated with sensorineural hearing impairment, but rarely is it associated with conductive or mixed deafness. A 22-year-old female presented with fusion of the cervical vertebrae, torticollis, scoliosis, pterygium colli, the Sprengel deformity with an omovertebral bone, concavity of the thorax and conductive hearing impairment of the right ear. Tympanotomy disclosed an atrophic long process of incus and a fixation of the stapes footplate, and stapedectomy was performed with immediate postoperative improvement of hearing. However, she developed a sudden hearing loss with dizziness soon after she had physical exercise on the 15th postoperative day, and revision surgery revealed a perilymph fistula of the oval window. Histological investigations of the removed stapes showed no specific osseous changes but hyperostosis of the posterior edge of the footplate. The literature is reviewed and the etiology of the conductive deafness and the perilymph fistula is discussed.

Pressure Gradients Affecting the Labyrinth during Hypobaric Pressure

1997 ◽  
Vol 106 (6) ◽  
pp. 495-502 ◽  
Author(s):  
Konrád S. Konrádsson ◽  
Björn I. R. Carlborg ◽  
Joseph C. Farmer
Keyword(s):  
Cerebrospinal Fluid ◽  
Eustachian Tube ◽  
Ambient Pressure ◽  
Pressure Gradients ◽  
Cochlear Aqueduct ◽  
Fluid Compartment ◽  
Large Pressure ◽  
Fluid Compartments ◽  
Pressure Changes ◽  
Considerable Pressure

Hypobaric effects on the perilymph pressure were investigated in 18 cats. The perilymph, tympanic cavity, cerebrospinal fluid, and systemic and ambient pressure changes were continuously recorded relative to the atmospheric pressure. The pressure equilibration of the eustachian tube and the cochlear aqueduct was studied, as well as the effects of blocking these channels. During ascent, the physiologic opening of the eustachian tube reduced the pressure gradients across the tympanic membrane. The patent cochlear aqueduct equilibrated perilymph pressure to cerebrospinal fluid compartment levels with a considerable pressure gradient across the oval and round windows. With the aqueduct blocked, the pressure decrease within the labyrinth and tympanic cavities was limited, resulting in large pressure gradients toward the chamber and the cerebrospinal fluid compartments, respectively. We conclude that closed cavities with limited pressure release capacities are the cause of the pressure gradients. The strain exerted by these pressure gradients is potentially harmful to the ear.

Congenital Cerebrospinal Otorrhea

1979 ◽  
Vol 88 (3) ◽  
pp. 358-365 ◽  
Author(s):  
Richard R. Gacek ◽  
Bruce Leipzig
Keyword(s):  
Cerebrospinal Fluid ◽  
Initial Segment ◽  
Congenital Defects ◽  
Facial Canal ◽  
Cerebrospinal Fluid Fistula ◽  
Fallopian Canal ◽  
Cochlear Aqueduct ◽  
Cerebrospinal Fluid Leaks ◽  
Fluid Fistula ◽  
Petromastoid Canal

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.

Body Mass Index and Imaging Abnormalities in Spontaneous Cerebrospinal Fluid Leaks of the Temporal Bone

2014 ◽  
Vol 151 (1_suppl) ◽  
pp. P80-P80
Author(s):  
Andrew M. Rivera ◽  
Daniel Jethanamest ◽  
Simon I. Angeli
Keyword(s):  
Body Mass Index ◽  
Cerebrospinal Fluid ◽  
Temporal Bone ◽  
Body Mass ◽  
Cerebrospinal Fluid Leaks

Three-dimensional temporal bone reconstruction from histological sections

2014 ◽  
Vol 128 (5) ◽  
pp. 416-420 ◽  
Author(s):  
N Ahmad ◽  
A Wright
Keyword(s):  
High Resolution ◽  
Temporal Bone ◽  
Personal Computer ◽  
Three Dimensional ◽  
Jugular Bulb ◽  
Chorda Tympani Nerve ◽  
Three Dimensional Model ◽  
Histological Sections ◽  
Volumetric Reconstruction ◽  
Stapes Footplate

AbstractObjective:To produce a high-resolution, three-dimensional temporal bone model from serial sections, using a personal computer.Method:Digital images were acquired from histological sections of the temporal bone. Image registration, segmentation and three-dimensional volumetric reconstruction were performed using a personal computer. The model was assessed for anatomical accuracy and interactivity by otologists.Results:An accurate, high-resolution, three-dimensional model of the temporal bone was produced, containing structures relevant to otological surgery. The facial nerve, labyrinth, internal carotid artery, jugular bulb and all of the ossicles were seen (including the stapes footplate), together with the internal and external auditory meati. Some projections also showed the chorda tympani nerve.Conclusion:A high-resolution, three-dimensional computer model of the complete temporal bone was produced using a personal computer. Because of the increasing difficulty in procuring cadaveric bones, this model could be a useful adjunct for training.

scholarly journals Erratum to “Surgical outcomes in the treatment of temporal bone cerebrospinal fluid leak: A systematic review” [Auris Nasus Larynx 45 (2018) 903–910]

2019 ◽  
Vol 46 (3) ◽  
pp. 483
Author(s):  
Federico Maria Gioacchini ◽  
Ettore Cassandro ◽  
Matteo Alicandri-Ciufelli ◽  
Shaniko Kaleci ◽  
Claudia Cassandro ◽  
...  
Keyword(s):  
Systematic Review ◽  
Cerebrospinal Fluid ◽  
Temporal Bone ◽  
Surgical Outcomes ◽  
Cerebrospinal Fluid Leak ◽  
Fluid Leak
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