Anomalies of the Ear in the Pierre Robin Triad

2005 ◽  
Vol 114 (8) ◽  
pp. 605-613 ◽  
Author(s):  
Philipp M. Gruen ◽  
Alfonso Carranza ◽  
Collin S. Karmody ◽  
Edgar Bachor
Keyword(s):  
Hair Cells ◽  
Semicircular Canals ◽  
Mastoid Process ◽  
Cochlear Hair Cells ◽  
Vestibular Aqueduct ◽  
Middle Ear Infection ◽  
Branchial Arches ◽  
Temporal Bones ◽  
Pierre Robin ◽  
Expected Position

Objectives: The Pierre Robin triad (PRT) consists of micrognathia-retrognathia, glossoptosis, and an oval or cleft palate. The goal of this study was to identify patterns of similarity to and differences from the two previous temporal bone studies of the PRT. Methods: Seven children with the PRT (ages, 45 minutes to 2 years; gestational ages, 41 to 43 weeks) were studied. Thirteen temporal bones were decalcified, sectioned at a thickness of 20 μm, and studied by light microscopy. Results: Our study demonstrated multiple architectural anomalies involving the entire ear, including abnormal auricles, and anomalies of the ossicles, including abnormal stapes footplates (6/13). All children showed signs of middle ear infection (12/13). Anomalies of the inner ear included aplasia of the lateral semicircular canals (5/13), a large vestibular aqueduct (2/13), and unusually large otoconia (1/13). In the mastoid process there were islands of cartilage in the expected position of Reichert's cartilage (9/13) and dehiscence of the fallopian canal (11/13). Loss of cochlear hair cells was seen in children who had antemortem hypoxia. Conclusions: Although the PRT is caused by various genes, most anomalies can be traced to the development of the first and second branchial arches.

Effect of Transverse Temporal Bone Fracture on the Fluid Compartment of the Inner Ear

1979 ◽  
Vol 88 (6) ◽  
pp. 741-748 ◽  
Author(s):  
Syed S. Rizvi ◽  
Kevin P. Gibbin
Keyword(s):  
Hair Cells ◽  
Endolymphatic Hydrops ◽  
Membranous Labyrinth ◽  
Temporal Bone Fracture ◽  
Complete Obstruction ◽  
Vestibular Hair Cells ◽  
Vestibular Aqueduct ◽  
Fluid Compartment ◽  
Temporal Bones ◽  
Transverse Fractures

Five temporal bones exhibiting transverse fractures were studied with a view to determining whether such fractures could lead to symptomatic endolymphatic hydrops. Four out of the five temporal bones showed the fracture line traversing the vestibular aqueduct. Two of these four showed complete obstruction of the endolymphatic duct. One of these had an intact membranous labyrinth and severe endolymphatic hydrops. The other had ruptures of the membranous labyrinth and no hydrops. Three ears exhibited partial preservation of auditory and vestibular hair cells and neurons. These findings are consistent with the concept that a transverse fracture may produce endolymphatic hydrops by obstructing the vestibular aqueduct while preserving enough audiovestibular epithelium and neurons to present as symptomatic Menière's disease.

Crosslinks between stereocilia in hair cells of the human and guinea pig vestibular labyrinth

1986 ◽  
Vol 100 (12) ◽  
pp. 1367-1374 ◽  
Author(s):  
David J. Jeffries ◽  
James O. Pickles ◽  
Michael P. Osborne ◽  
Peter H. Rhys-Evans ◽  
Spiro D. Comis
Keyword(s):  
Guinea Pig ◽  
Hair Cells ◽  
Semicircular Canals ◽  
Sensory Transduction ◽  
Hair Bundle ◽  
Vestibular Labyrinth ◽  
Vestibular Hair Cells ◽  
Cuticular Plate ◽  
Temporal Bones

AbstractThe saccules and ampullae of the semicircular canals from human and guinea pig temporal bones were fixed in glutaraldehyde without osmium. Crosslinks were seen between stereocilia of the vestibular hair cells, similar to those previously demonstrated in the guinea pig, although an additional set of crosslinks was displayed: first, horizontal crosslinks were seen between adjacent stereocilia, occupying most of the length of the hair bundle; secondly, a single upward-pointing link ran from the apex of each shorter stereocilium into the shaft of the adjacent taller ster-eocilium; thirdly, an extensive array of horizontal links were demonstrated between stereocilia close to their insertion into the cuticular plate. We suggest that these basal crosslinks support the long vestibular stereocilia rendering them more rigid, and that the upwind pointing crosslinks are responsible for the initiation of sensory transduction.

Temporal Bone Histopathological Findings in Trisomy 13 Syndrome

1975 ◽  
Vol 84 (21_suppl) ◽  
pp. 3-20 ◽  
Author(s):  
Isamu Sando ◽  
Alberto Leiberman ◽  
LaVonne Bergstrom ◽  
Soji Izumi ◽  
Raymond P. Wood
Keyword(s):  
Facial Nerve ◽  
Middle Ear ◽  
Semicircular Canals ◽  
Trisomy 13 ◽  
Obtuse Angle ◽  
Fetal Life ◽  
Horizontal Canal ◽  
Histopathological Findings ◽  
Middle Ear Infection ◽  
Temporal Bones

This study reports the histopathological findings of 14 temporal bones from infants with trisomy 13 syndrome. The most primitive anomalies in the structures of the inner and middle ears in the present series are those of the semicircular canals, particularly of the horizontal canals: flattened horizontal canal cristae, absence or opening of the utricular endolymphatic valve, small facial nerve, and obtuse angle of the geniculate area of the facial nerve. Each ear demonstrated more than one of those anomalies. The anomalies present features similar to those found in the structures of the normal six to ten-week fetus. Many other mild anomalies observed appear to demonstrate features similar to those seen in the same structures in later fetal life. Reviewing these findings, most of the anomalies that were found in the inner and middle ears appear to be the result of poor development of the structures for reasons which are now unclear. In addition, middle ear infection was found in all cases.

Vein of the Vestibular Aqueduct

1979 ◽  
Vol 88 (6) ◽  
pp. 759-767 ◽  
Author(s):  
Antonio Mazzoni
Keyword(s):  
Semicircular Canals ◽  
Posterior Wall ◽  
Jugular Bulb ◽  
Clear Fluid ◽  
Vestibular Aqueduct ◽  
Stereo Microscope ◽  
Capillary Bed ◽  
The Rich ◽  
Temporal Bones ◽  
Vascular Injection

The vein of the vestibular aqueduct (VVA) was investigated in a series of 40 human temporal bones. The processing included vascular injection with a colored medium, decalcification and cutting in serial, thick sections, which were put in a clear fluid and studied with a stereo-microscope. The labyrinthine roots of the VVA are the single veins of the ampulla and simple limbs of the semicircular canals and of the posterior wall of the utricle. They drain the rich capillary bed of the simple endolymphatic walls of the canals and the utricle, as well as a small peripheral area of the cristae and the utricular macula. The VVA leaves the vestibule through an individual bone canal running parallel to the vestibular aqueduct up to the dura of the posterior side of the petrosa in the area of the endolymphatic sac. It then opens in the inferior petrosal sinus or the jugular bulb. The vein receives other branches from the bone, dura and sac. Correct information on the course of this vein appears to be lacking in contemporary textbooks and articles, although it has been correctly described since the last century.

Arterial Supply of the Human Endolymphatic Duct and Sac

1993 ◽  
Vol 108 (2) ◽  
pp. 141-148 ◽  
Author(s):  
Arun K. Gadre ◽  
Jose N. Fayad ◽  
Michael J. O'Leary ◽  
Rizkalla Zakhary ◽  
Fred H. Linthicum
Keyword(s):  
Sigmoid Sinus ◽  
Mastoid Process ◽  
Occipital Artery ◽  
Arterial Blood Supply ◽  
Vascular Casts ◽  
Vestibular Aqueduct ◽  
Arterial Blood ◽  
Endolymphatic Duct ◽  
Temporal Bones ◽  
Arterial Anatomy

The arterial anatomy of the endolymphatic duct and sac was studied in vascular casts of methyl methacrylate of six human heads. The chief source of arterial blood supply to the endolymphatic duct and sac appeared to be the occipital artery. Arterioles entered the bone of the mastoid process. Arterioles in bone, the walls of the sigmoid sinus, and the posterior fossa dura coursed medially to supply the endolymphatic sac. The orientation of arterioles tended to be along the long axis of the endolymphatic duct and sac, whereas venules were more likely to be circumferentially oriented. Arterioles arising from dural vessels divided into deeper branches, which supplied periductal connective tissue, and superficial branches, which entered canaliculi of the vestibular aqueduct. Gross anatomic findings were confirmed by histologic examination of temporal bones.)

Temporal Bone Histopathological Findings in Trisomy 13 Syndrome

1975 ◽  
Vol 84 (4_suppl) ◽  
pp. 2-20 ◽  
Author(s):  
Isamu Sando ◽  
Alberto Leiberman ◽  
LaVonne Bergstrom ◽  
Soji Izumi ◽  
Raymond P. Wood
Keyword(s):  
Facial Nerve ◽  
Middle Ear ◽  
Semicircular Canals ◽  
Trisomy 13 ◽  
Obtuse Angle ◽  
Fetal Life ◽  
Horizontal Canal ◽  
Histopathological Findings ◽  
Middle Ear Infection ◽  
Temporal Bones

This study reports the histopathological findings of 14 temporal bones from infants with trisomy 13 syndrome. The most primitive anomalies in the structures of the inner and middle ears in the present series are those of the semicircular canals, particularly of the horizontal canals: flattened horizontal canal cristae, absence or opening of the utricular endolymphatic valve, small facial nerve, and obtuse angle of the geniculate area of the facial nerve. Each ear demonstrated more than one of those anomalies. The anomalies present features similar to those found in the structures of the normal six to ten-week fetus. Many other mild anomalies observed appear to demonstrate features similar to those seen in the same structures in later fetal life. Reviewing these findings, most of the anomalies that were found in the inner and middle ears appear to be the result of poor development of the structures for reasons which are now unclear. In addition, middle ear infection was found in all cases.

scholarly journals Current Response in CaV1.3–/– Mouse Vestibular and Cochlear Hair Cells

2021 ◽  
Vol 15 ◽  
Author(s):  
Marco Manca ◽  
Piece Yen ◽  
Paolo Spaiardi ◽  
Giancarlo Russo ◽  
Roberta Giunta ◽  
...  
Keyword(s):  
Hair Cells ◽  
Signal Transmission ◽  
Basolateral Membrane ◽  
Semicircular Canals ◽  
Type I ◽  
Current Response ◽  
Type Ii ◽  
Current Profile ◽  
Cochlear Hair Cells ◽  
Vestibular Hair Cells

Signal transmission by sensory auditory and vestibular hair cells relies upon Ca2+-dependent exocytosis of glutamate. The Ca2+ current in mammalian inner ear hair cells is predominantly carried through CaV1.3 voltage-gated Ca2+ channels. Despite this, CaV1.3 deficient mice (CaV1.3–/–) are deaf but do not show any obvious vestibular phenotype. Here, we compared the Ca2+ current (ICa) in auditory and vestibular hair cells from wild-type and CaV1.3–/– mice, to assess whether differences in the size of the residual ICa could explain, at least in part, the two phenotypes. Using 5 mM extracellular Ca2+ and near-body temperature conditions, we investigated the cochlear primary sensory receptors inner hair cells (IHCs) and both type I and type II hair cells of the semicircular canals. We found that the residual ICa in both auditory and vestibular hair cells from CaV1.3–/– mice was less than 20% (12–19%, depending on the hair cell type and age investigated) compared to controls, indicating a comparable expression of CaV1.3 Ca2+ channels in both sensory organs. We also showed that, different from IHCs, type I and type II hair cells from CaV1.3–/– mice were able to acquire the adult-like K+ current profile in their basolateral membrane. Intercellular K+ accumulation was still present in CaV1.3–/– mice during IK,L activation, suggesting that the K+-based, non-exocytotic, afferent transmission is still functional in these mice. This non-vesicular mechanism might contribute to the apparent normal vestibular functions in CaV1.3–/– mice.

Fluorocitrate Neural Dystrophy of the Guinea Pig Cochlea

1975 ◽  
Vol 33 ◽  
pp. 368-369
Author(s):  
G.J. Spector ◽  
C.D. Carr ◽  
I. Kaufman Arenberg ◽  
R.H. Maisel
Keyword(s):  
Hearing Loss ◽  
Hair Cells ◽  
Sodium Phosphate ◽  
Sensory Cells ◽  
Barium Salt ◽  
Perfusion Medium ◽  
Cochlear Hair Cells ◽  
Neural Degeneration ◽  
Sodium Phosphate Buffer ◽  
Cochlear Neurons

All studies on primary neural degeneration in the cochlea have evaluated the end stages of degeneration or the indiscriminate destruction of both sensory cells and cochlear neurons. We have developed a model which selectively simulates the dystrophic changes denoting cochlear neural degeneration while sparing the cochlear hair cells. Such a model can be used to define more precisely the mechanism of presbycusis or the hearing loss in aging man.Twenty-two pigmented guinea pigs (200-250 gm) were perfused by the perilymphatic route as live preparations using fluorocitrate in various concentrations (15-250 ug/cc) and at different incubation times (5-150 minutes). The barium salt of DL fluorocitrate, (C6H4O7F)2Ba3, was reacted with 1.0N sulfuric acid to precipitate the barium as a sulfate. The perfusion medium was prepared, just prior to use, as follows: sodium phosphate buffer 0.2M, pH 7.4 = 9cc; fluorocitrate = 15-200 mg/cc; and sucrose = 0.2M.

Further studies on the ultrastructure of the cochlea

1990 ◽  
Vol 48 (3) ◽  
pp. 440-441
Author(s):  
Zhixian Wang ◽  
Pinjin Zhu ◽  
Jianhe Sun ◽  
Xuezheng Song
Keyword(s):  
Hair Cells ◽  
Military Medicine ◽  
Outer Hair Cells ◽  
Apical Surface ◽  
Epithelial Surface ◽  
Hearing Research ◽  
New Findings ◽  
Fine Structures ◽  
Temporal Bones ◽  
Accurate Observation

Hearing research is important not only for clinical, professional and military medicine, but also for toxicology, gerontology and genetics. Ultrastructure of the cochlea attracts much attention of electron microscopists, (1―3) but the research lags far behind that of the other parts of the organnism. On the basis of careful microdissection, technical improvment and accurate observation, we have got some new findings which have not been reported in the literature.We collected four cochleas from human corpses. Temporal bones dissected 1 h after death and cochleas perfused with fixatives 4 h after death were good enough in terms of preservation of fine structures. SEM:The apical surface of OHCs (Outer hair cells) and DTs (Deiters cells) is narrower than that of IPs (Inner pillar cells). The mosaic configuration of the reticular membrane is not typical. The stereocilia of IHCs (Inner hair cells) are not uniform and some kinocilia could be seen on the OHCs in adults. The epithelial surface of RM (Reissner’s membrane) is not smooth and no mesh could be seen on the mesothelial surface of RM. TEM.

Pengaruh sisplatin dosis tinggi terhadap penurunan fungsi sel rambut luar koklea

2013 ◽  
Vol 40 (2) ◽  
Author(s):  
Asti Kristianti ◽  
Teti Madiadipoera ◽  
Bogi Soeseno
Keyword(s):  
Hair Cells ◽  
Malignant Neoplasm ◽  
Otoacoustic Emission ◽  
Outer Hair Cells ◽  
High Dose ◽  
Distortion Product Otoacoustic Emission ◽  
Inclusion Criteria ◽  
Cochlear Hair Cells ◽  
Distortion Product ◽  
Bilateral Sensorineural Hearing Loss

Background: Chemotherapy is worldwide used nowadays, and its toxicity still remain a problemespecially toxicity to the ear (ototoxicity). Cisplatin (cis-diamminedichloroplatinum) is one of themost commonly used chemotherapy and highly potent in treating epithelial malignancies. Ototoxicitycaused by cisplatin is irreversible, progressive, bilateral, sensorineural hearing loss especially on highfrequency (4-8 KHz) accompanied by tinnitus. Purpose: To observe the cochlear outer hair cells damagein malignancies patients treated with cisplatin. Methods: This study is an observational analytic studywith prospective design to determine the influence of high dose cisplatin on cochlear outer hair cellsfunction. The research was carried out at the ENT-HNS Department, Hasan Sadikin General HospitalBandung, from November 2007 until June 2008. Audiometry, tympanometry, and distortion productotoacoustic emission (DPOAE) examinations were conducted before chemotherapy and DPOAE, andtimpanometry was again measured three days after first and second cycles of cisplatin administration. McNemar test was performed to calculate the effects of high-dose cisplatin to the cochlear outer haircells function. To compare pre and post-cisplatin on alteration of cochlear hair cells function, Wilcoxontest was used. Results: In this study 60 ears from 30 subjects that meet the inclusion criteria, consistedof 25 man (83.3%) and 5 women (16.7%). The prevalence of damaged cochlear outer hair cells were63% at first cycle and 70% at second cycle of cisplatin administration. The decline of cochlear outerhair cells function was significant (p<0.001). Conclusion: High-dose cisplatin decreases cochlear outerhair cells function in patients with malignant neoplasm. Abstrak : Latar belakang: Kemoterapi sekarang rutin digunakan secara klinis di seluruh dunia. Sejalan denganhal tersebut toksisitas kemoterapi, khususnya terhadap telinga saat ini menjadi perhatian. Sisplatin(cis-diamminedichloroplatinum) adalah salah satu obat kemoterapi yang paling banyak digunakandan paling manjur untuk terapi keganasan epitelial. Efek ototoksik sisplatin yaitu terjadi gangguandengar sensorineural yang irreversible, progresif, bilateral pada frekuensi tinggi (4-8 kHz), dan disertaidengan tinitus. Tujuan: Untuk menilai penurunan fungsi sel rambut luar koklea pada penderita tumorganas sesudah pemberian sisplatin dosis tinggi dengan menggunakan DPOAE. Metode: Studi analitikobservasional dengan rancangan prospektif di Bagian IK. THT-KL RS. Hasan Sadikin Bandung mulaibulan November 2007 sampai dengan Juni 2008. Pada penelitian ini dilakukan pemeriksaan audiometrinada murni, timpanometri, dan distortion product otoacoustic emission (DPOAE) prakemoterapi, kemudianDPOAE dan timpanometri diulang tiga hari sesudah siklus pertama dan kedua kemoterapi sisplatin. Datayang diperoleh diuji dengan uji McNemar dan uji Wilcoxon. Hasil: Dari penelitian didapat 60 telingadari 30 subjek penelitian yang memenuhi kriteria inklusi yang terdiri dari 25 laki-laki (83,3%) dan 5perempuan (16,7%). Insidens penurunan fungsi sel rambut luar koklea sebesar 63% (38 kasus) sesudahsiklus pertama dan 70% (42 kasus) sesudah siklus kedua. Hubungan penurunan fungsi sel rambut luarkoklea memberikan nilai yang sangat bermakna sejak pemberian siklus pertama (p<0,001). Kesimpulan:Pemberian sisplatin dosis tinggi pada penderita tumor ganas menyebabkan penurunan fungsi sel rambutluar koklea.Kata kunci: kemoterapi, sisplatin dosis tinggi, sel rambut luar koklea.

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