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Biomedical subjects

I Sando

Publications and source records attributed to I Sando.

At least 19 recordsLinked to original sources

Branchio-oto-renal syndrome: further delineation of an underdiagnosed syndrome.

We report on a woman who was diagnosed with branchio-oto-renal (BOR) syndrome after 2 pregnancies complicated by oligohydramnios due to renal hypoplasia and agenesis. Both babies died neonatally of pulmonary hypoplasia. Histopathology of the temporal bones of the second child showed marked immaturity of the middle ear cleft, ossicles, facial nerve and canal, and cochlear nerve. Maternal renal ultrasound study was normal although intravenous pyelography indicated renal hypoplasia. The frequency of BOR syndrome among cases of recurrent fetal renal hypoplasia/dysplasia or agenesis is unknown, and parental renal ultrasonography may not identify a heritable renal defect. Investigations should include a family history, and examination of relatives to look for preauricular pits, lacrimal duct stenosis, and branchial fistulae and/or cysts. Hearing studies and IVP may be indicated.

Abnormalities, Multiple

Three-dimensional computer-aided reconstruction and measurement of the temporal bone for singular neurectomy.

A computer reconstruction method was used to study five normal temporal bones for the three-dimensional anatomy necessary for singular neurectomy. The length of the singular canal was 4.9 +/- 0.6 mm. The distal portion of the singular canal courses at a sharp angle (48.2 degrees) to the direction of the transmeatal surgical approach for this operation. The reconstructions indicated that the singular canal can be exposed by introducing a drill through the external auditory meatus at a point 0.71 mm posteroinferior to the posteromedial margin of the round window on the saucerized medial wall of the round window niche. The distance from this site to the distal end-portion of the singular canal averaged 1.47 mm. The point on the drilling course (from the drilling site to the singular canal) closest to the vestibular end of the hook portion of the cochlea was 0.48-1.00 mm from the drilling site. The reconstructions also indicated that the dissection should proceed superomedially, if necessary, after a 1-mm-deep straight dissection along the transmeatal approach. The ampulla of the posterior semicircular canal, cochlear aqueduct and inferior cochlear vein were all found to lie within 2 mm of the drilling point.

Adolescent

Stria vascularis in Ménière's disease: a quantitative histopathological study.

To investigate the role of pathology of the stria vascularis in Ménière's disease, the vascularity and the cross-sectional area of the stria vascularis in a midmodiolar section of the cochlea were examined by histological observation and a computer-aided planimetric, respectively, in eight temporal bones from individuals with Ménière's disease and eight age-matched normal temporal bones. The number of vessels in the stria vascularis was significantly smaller in most of the cochlear turns in ears with Ménière's disease than in control ears, and the cross-sectional area of the stria vascularis in the same sections was significantly smaller in all the cochlear turns in ears with Ménière's disease than in controls. Furthermore, vascularity correlated significantly with the cross-sectional area of the stria vascularis in all the temporal bones examined. From these results it appears that poor vascularity of the stria vascularis is closely related to strial atrophy, and that these pathological findings might be important factors in the pathophysiology of Ménière's disease.

Adult

Temporal bone histopathological findings in campomelic dysplasia.

Both 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 cochlea and 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.

Abnormalities, Multiple

Elastin at the hinge portion of the eustachian tube cartilage in specimens from normal subjects and those with cleft palate.

The density of elastin in the intermediate portion between the lateral lamina and the medial lamina of human eustachian tube (ET) cartilage was examined in six normal adults, seven normal children, and six children with cleft palate (CP) in order to obtain information about how the physical properties of the ET cartilage differ as a function of age and presence of CP. Cross sections of the midcartilaginous portions of the ETs that had been stained with Weigert's elastin stain were photographed at uniform magnification, the area for study was projected, and the meshlike are of the ET cartilage that stained elastin-positive was represented on paper by lines. A digitizer was used to measure the total length of all the lines representing elastin in each photomicrograph, and the mean was determined for each of the three groups. The mean density of elastin was significantly greater in normal adults than in normal children (Student's t test, t = 2.781; p less than .02). It was also significantly greater in normal children than in CP children (Wilcoxon t = 24.0; p less than .05). These results appear to indicate that CP children have poorer elasticity in this area of the ET cartilage, which might cause functional obstruction (floppiness) of the ET in those children.

Adult

Distributions of eustachian tube goblet cells and glands in children with and without otitis media.

The distributions of the goblet cells and submucosal glands in eustachian tubes (ETs) from children with or without otitis media (OM) were studied. From 12 temporal bone-ET specimens obtained from children 1 to 7 years old at death, representative vertically cut histology sections were selected, stained with periodic acid-Schiff-Alcian blue and hematoxylin, and studied by light microscopy. In children without OM, the density of the goblet cells was significantly lower in the roof than in the floor of the ET and was highest in the midcartilaginous portion; glands were most richly distributed near the pharyngeal orifice, and became less densely distributed toward the tympanic portion. In children with OM, the density of the goblet cells was significantly higher both in the roof and floor portions, and the glands were more richly distributed than in the ETs of children without OM. The possibility that the observed increase in ET secretory components in children with OM impairs the passage of air through these tubes is discussed.

Acute Disease

Histopathology of tubotympanum of children with otitis media treated with ventilation tubes.

We studied histopathologically the middle ear (ME) and eustachian tube (ET) in 12 temporal bones from eight children who had had otitis media with effusion that had been treated with ventilation tubes (VTs) for 3 weeks to 11 months. In the ME, 1) only a minimal amount of effusion was seen in most of the ears treated for 3 months or longer, 2) the severity of submucosal inflammation almost paralleled the amount of effusion, 3) the amount of granulation tissue on the tympanic membrane at the site of VT insertion also seemed to parallel the degree of ME inflammation, and 4) epidermal ingrowth was seen in both of the two ears treated with VTs for 10 and 11 months. Pathologic findings in the ET were generally more severe than those in the ME, but tended to decrease with longer VT therapy. In two ears with VTs obstructed by inflammatory granulomatous tissue, the entire ME cavity was filled with effusion, and dense effusion plugged the bony portion of the ET. The results of this study indicate that 3 to 11 months of treatment with a VT is effective for reversing the ME effusion and for reducing inflammation in the ME. Some inflammation may be left in the ET, however, even with 11 months of VT therapy, and epithelial ingrowth should be watched for when VT therapy lasts 10 months or longer. Despite the fact that most of our temporal bones were from infants and children who had a variety of systemic diseases or disorders and in whom their terminal event might have contributed to our findings, we consider these temporal bones to be informative.

Child

Facial canal dehiscence: histologic study and computer reconstruction.

The incidence, location, shape, and dimensions of dehiscences in the facial canal to the middle ear space were studied in 160 temporal bones obtained from 129 individuals 24 gestational weeks to 109 years of age at death by means of light microscopy and our computer reconstruction and measurement method. Dehiscences were observed in 95 of the 129 individuals (74%) and in 119 of the 160 bones (74%). The incidence was found to be quite high among fetuses and newborns, lowest in individuals in their twenties and then again quite high in the geriatric population (chi 2 test, chi 2 = 5.45 and 4.41, p < .05). The most frequent site of dehiscence was the oval window area, particularly in its posterior half (57% of all ears) on the inferior to inferomedial aspects of the canal; these dehiscences were clearly demonstrated in reconstructed images. The incidence of dehiscence in the area of the cochleariform process was 16%, and all these dehiscences were on the lateral to superolateral aspect of the canal. The second genu area and the mastoid portion were sites of dehiscence in 21% and 18%, respectively, of specimens; more than half of the dehiscences in the second genu area and mastoid portion were on the lateral to anterolateral and posterior aspects of the facial canal, respectively. The shape of the dehiscence tended to be oval in the oval window area, but rather irregular in the other areas. The dehiscences ranged from 0.4 to 2.64 mm in length, from 0.12 to 1.59 mm in width, and from 0.03 to 1.87 mm2 in surface area. The proximity of these dehiscences to the field of otologic surgery is stressed.

Adolescent

Computer-aided three-dimensional measurement of the human vestibular apparatus.

Using a computer-aided three-dimensional reconstruction and measurement method, 12 measurements were made to determine the dimensions of the maculae, cristae ampullares, and semicircular canals in 18 temporal bones from nine pairs of age-matched male and female individuals (1 day to 76 years old). The surface areas of the utricular and saccular maculae were significantly larger in male than in female specimens (two-way analysis of variance, F = 9.00, df = 1, p less than 0.01; F = 4.57, df = 1, p less than 0.05, respectively). The width of the utricular macula and the length of the saccular macula were also significantly greater in male than in female specimens (two-way analysis of variance, F = 5.17, df = 1, p less than 0.05; F = 4.33, df = 1, p less than 0.05, respectively). Finally, the three semicircular canals were larger in diameter in male vs. female specimens; this difference was statistically significant for the superior semicircular canal (two-way analysis of variance, F = 10.74, df = 1, p less than 0.01). By contrast, none of these dimensions of those vestibular structures showed any significant change in size with advancing postnatal age. We propose from these findings that there appears to be sexual dimorphism in the vestibular apparatus.

Adolescent

Temporal bone histopathologic findings of Waardenburg's syndrome: a case report.

A histopathological study of the temporal bones of a 3-year-old black girl who had bilateral deafness associated with Waardenburg's syndrome type II showed a similar pattern of pathology in both ears. The most striking findings were an absence of pigmentation in the inner ear and cochleosaccular abnormality. This is, to our knowledge, only the third report on human temporal bone histopathology in Waardenburg's syndrome and the first report of such a case with absence of pigment (melanin) in the inner ear. A possible association of hearing loss with absence of inner ear pigment in this case is discussed.

Child, Preschool

Three-dimensional anatomy of human Scarpa's ganglion.

Using a computer-aided three-dimensional reconstruction and measurement method, the authors studied the shape and dimensions of Scarpa's ganglion and interrelations of the ganglion to nearby important anatomical structures in 10 normal human temporal bones obtained from 10 individuals of different ages (12 gestational weeks to 72 years). In the postnatal cases, Scarpa's ganglion was shaped like a distorted hourglass. Although the shape of the ganglion varied somewhat among specimens, a superior division, inferior division, and isthmus ganglionaris were distinguishable in each case. Volume of the ganglion was relatively consistent after birth, so that width and height of the ganglion were inversely correlated with each other (linear regression analysis, P less than .05). There seemed to be, however, a possible postnatal increase in the distances from the ganglion to neighboring structures and in the length of the internal auditory canal; these increases were probably the result of postnatal growth of the temporal bone. Moreover, the width of the ganglion in any single specimen was found to correlate significantly positively with the distances from the ganglion to the vestibular end-organs and the length of the internal auditory canal in that specimen (linear regression analysis, P less than .01). This finding suggests that Scarpa's ganglion may be elongated secondary to the elongation of the vestibular nerve due to the growth of the temporal bone. In fetuses, each portion of the ganglion was less clearly identifiable than that portion in postnatal cases. The dimensions of Scarpa's ganglion were found to have reached maturity around the time of birth.

Adolescent

Three-dimensional surgical anatomy for stapes surgery computer-aided reconstruction and measurement.

To define anatomical relationships relevant to stapes surgery, computer-aided three-dimensional reconstruction and measurement were performed on nine normal temporal bones. The mean distance from the inferior portion of the long process of the incus to the center of the oval window was 3.80 mm. The shortest distance from the center of the oval window to the utricular macula, saccular membrane, and macula averaged 1.37, 1.60, and 2.13 mm. Surgery directed posteromedial-superior from the oval window was found to be most dangerous because it would come so close to the utricular macula; a posteromedial-inferior approach was found to be safest. The distance from the inferior margin of the oval window inferiorly to the cochlear duct in the hook portion ranged between 0.58 and 1.29 mm, suggesting that when a drill hole is made on the inferior margin of the oval window to lift up a depressed stapes footplate, the hole should not be greater than 0.5 mm in diameter.

Adolescent

Dark cell pathology in Menière's disease.

The dark cells in the cristae of the semicircular canals were studied histopathologically in 9 temporal bones from individuals with Menière's disease (MD group), 7 temporal bones with endolymphatic hydrops from individuals without a history of Menière's disease (non-MD hydrops group), and 10 normal temporal bones (control group). The density of the dark cells was significantly lower in specimens in the MD group versus the non-MD hydrops group (Wilcoxon's ranking test, t = 90.5, p less than 0.01) and control group (Wilcoxon's ranking test, t = 50.0, p less than 0.01), and many of the dark cells were found to be abnormal in the specimens from the MD group. The difference in dark cell density between the non-MD hydrops group and control group, however, was not significant (Wilcoxon's ranking test, t = 75.0), and few cells in these groups were abnormal. It is speculated that the differences in density and the abnormalities in dark cell morphology might be either factors in the etiology of endolymphatic hydrops or results of Menière's disease. Other, unknown, factors must be postulated to produce endolymphatic hydrops in ears with hydrops but without MD.

Adult

Computer-aided three-dimensional reconstruction and measurement of microfissures.

The spatial anatomy of microfissures in the round and oval window areas was investigated in 24 randomly selected normal human temporal bones by our computer-aided three-dimensional reconstruction and measurement method. Microfissures that communicated with the middle ear surface in the round window area were seen in 21 cases (87%) and were located on the posteromediosuperior aspect of the round window niche; whereas microfissures in the oval window area, seen in seven cases (29%), were located at various sites, but mostly in the area below the oval window. Of the 21 microfissures seen in the round window niche, only four were found to be visible through the aperture of the round window niche when viewed from the lateral direction. The mean lengths of the microfissures seen on the middle ear surface in the round and oval window areas were 0.95 +/- 0.66 mm and 0.74 +/- 0.44 mm, respectively. The microfissures in the round window area were significantly longer in individuals 20 years of age and older, than in those less than 20 years (Wilcoxon test, t = 21.5, p less than 0.01); this tendency for microfissures to be longer in older subjects was also found for microfissures in the oval window area. The possible clinical and pathologic significance of these results is discussed.

Adolescent

Vestibular aqueduct in Menière's disease and non-Menière's disease with endolymphatic hydrops: a computer aided volumetric study.

The volume of the vestibular aqueduct was studied by a computer-aided volumetric method in 9 temporal bones with endolymphatic hydrops from individuals with Menière's disease (MD), 7 temporal bones with endolymphatic hydrops from individuals without a history of Meniere's disease (non-MD hydrops), and 10 normal temporal bones (controls) to investigate the cause of endolymphatic hydrops in both MD and non-MD hydrops. A hypoplastic vestibular aqueduct was found significantly more often in the MD group than in either the non-MD hydrops group (chi 2-test, chi 2 = 4.063, p less than 0.05) or the control group (chi 2-test, chi 2 = 6.363, p less than 0.05). The difference in volume between the non-MD hydrops group and the control group was not significant. It is speculated that a small vestibular aqueduct (presumably containing a small endolymphatic sac) might be a predisposing factor in Menière's disease. In contrast, in non-MD hydrops, there seems to be no correlation of endolymphatic hydrops with a hypoplastic vestibular aqueduct and endolymphatic sac.

Adult

Abnormalities of lateral cartilaginous lamina and lumen of eustachian tube in cases of cleft palate.

Development of the lateral lamina (LL) of the eustachian tube (ET) cartilage was investigated in cleft palate (CP) cases and controls by measuring the areas of the LL and medial lamina (ML) of the ET cartilage in histologic cross section, and comparing the ratio of the area of LL to that of ML (LL/ML) in CP cases and controls. The degree to which the ET lumen appeared straight or C-shaped in cross section was also measured. Thirty temporal bones with the entire ET--10 CP cases and 20 controls--were studied. The ratio of the areas of the ET cartilage was significantly smaller (t = 2.903, p less than .01) and the curvature of the ET lumen was significantly less (t = 2.080, p less than .05; chi 2 = 3.936, p less than .05) in CP cases than in controls. In addition, in all 30 CP cases and controls, the ratio of the areas of cartilage was significantly less in specimens with straight ET lumens than in those with C-shaped lumens (t = 2.713, p less than .02). The results appear to show that the ET cartilages and lumens are anomalous in CP cases. These anomalies are considered to be important factors in active ventilatory dysfunction of the ET.

Cartilage

Sexual dimorphism and development of the human cochlea. Computer 3-D measurement.

The length of the cochlear turns was measured in 9 pairs of temporal bones from age-matched male and female individuals (1 day to 76 years old), using a computer-aided three-dimensional reconstruction and measurement method. The mean cochlear length was significantly longer (Two-way analysis of variance, F = 31.87, d.f. = 1, p less than 0.01) in males (37.1 +/- 1.6 mm) than in females (32.3 +/- 1.8 mm), whereas it did not vary with postnatal age in either sex. Sexually dimorphic cochlear length may pose a new issue in auditory physiology in man. The lack of postnatal elongation also indicates that length of the cochlea becomes close to its maximum during fetal life.

Aging