Ann 0101 88 :1979
TEMPORAL BONE ANOMALIES ASSOCIATED WITH CONGENITAL HEART DISEASE TETSUYA EGAMI, MD, DMS ISAMU SANDO, MD, DMS
EUGENE N. MyERS, MD, FACS
PITTSBURGH, PENNSYLVANIA
A temporal bone histopathological study was conducted to detect temporal bone anomalies in 20 cars (10 cases) of individuals with congenital heart anomalies. We restricted our study to patients more than one year of age, and to heart anomalies of unknown etiology. The temporal bones were obtained from refrigerated cadavers, fixed in formalin, embedded in celloidin, cut in a horizontal plane, stained with H & E, and mounted on glass slides for light microscopic study. Anomalies observed in the middle ear were: remnants of mesenchymal tissue (8 ears), wide angle of the facial genu (6 cars), persistence of the stapedial artery (5 ears), large defect of the facial canal (4 ears), high jugular bulb (4 ears), and bulky incus (2 cars). Inner ear anomalies consisted of a shortened cochlea (5 ears), anomaly of the horizontal canal (3 ears), anomaly of the posterior canal (2 ears), obliteration of the cochlear aqueduct (2 ears), and patent utriculoendolymphatic valve (1 ear). Most of the anomalies observed appeared to be due to arrested development, and resembled features which may be found at various stages of fetal life. Structural anomalies were more commonly found in the mesoderm than in the ectoderm, and middle ear anomalies were more frequently encountered than anomalies of the inner ear. No definite relationship between these anomalies observed in the temporal bone and hearing problems which had been recorded clinically for these patients could be detected. However, the large defect (more than one third of the circumference) of the facial canal, the high jugular bulb, and the stapedial artery persistence should be recognized as problems since they may be encountered during middle ear surgery.
Congenital anomalies of the temporal findings of temporal bone histopathobone have been reported to occur in logic studies of such patients who lived association with other anomalies.' For longer than one year and, where posinstance, Kelemen- reported on the oc- sible, to emphasize the clinical sigcurrence of anomalies of the temporal nificance of these findings. bone in neonates and suggested that MATERIALS AND METHODS ear anomalies may coexist with congenital heart disease in some patients. This study made use of 20 temporal bones However, anomalies of the ear associ- from ten cases. The cases included only patients of more than one year of age whose maated with congenital heart disease, but jor significant problem was the presence of not associated with any known syn- cardiac anomalies. The temporal bones were drome or disease, have not been suf- removed from refrigerated cadavers, fixed in formalin, decalcified, embedded in celloidin, ficiently investigated. Recent developments in cardiac surgery have given congenital heart disease patients a prolonged life span. As a result, more associated ear problems may be encountered in these individuals than previously, including ear anomalies which require surgical intervention. The purpose of this paper is to report the
and sectioned horizontally at 20.u thickness. Every tenth section was stained with H & E and mounted on a glass slide for light microscopic study. CASE HISTORIES
Summaries of the ear histories, of the heart abnormalities, and of associated general anomalies which pertain to these cases are tabulated in Table 1,
From the Departments of Otolaryngology and Pathology, University of Pittsburgh School of Medicine, Eye and Ear Hospital. Pittsburgh, Pennsylvania; and the Department of Otolaryngology, University of COlorado Medical Center. Denver. Colorado. This study was supported In part by Research Grants NS 09293-05, NS 13787-01, and NS 12748-02 from the National Institute of Neurological and Communicable Diseases and Stroke.
12
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TEMPORAL BONE ANOMALIES & HEART DISEASE
73
TABLE 1. SUMMARIES OF EAR HISTORIES, CARDIAC ABNORMALITIES AND ASSOCIATED GENERAL ANOMALIES IN TEN CASES Case
Age
Sex
Ear History
Associated Abnormalities
Cardiac Abnonnality
1
12 mo
M
None
2
22 mo
M
None
3
3 yr
F
None
4
9yr
F
None
5
12 yr
F
None
6
13 yr
F
None
7
17 yr
M
None
8
20 yr
M
Lt. profound deafness
VSD, PS, surgery at age 8 yr
9
26 yr
F
VSD,PDA
10
35 yr
M
Bilateral SND (60 dB at 8 kHz) OMC with conductive deafness followed by Jrofound deafness ter ototoxic drug administration
ASD, died after cardiac surgery Transposition of great vessels, VSD Tetralogy of FaIlot, died after cardiac surgery A-V canal, died after cardiac surgery Tetralogy of FaIlot, surgery at age 5 yr A-V canal, transposition of great vessels ASD, VSD, PS, A-V canal, anomalous pulmcnary venous return
Tetralogy of FaIlot, surgery at age 12, died after second cardiac operation
Cleft soft palate None Duodenal atresia, Scleral dermoid Meckel's diverticulum None None Absence of spleen, supernumerary pulmonary lobulation Absence of right kidney and right testis Scoliosis and lordosis Periventricular brain cyst
ASD - Atrial septal defect; A-V canal - Atria-ventricular canal: OMC - Otitis media chronlea; PDA - Patent ductus arteriosus; PS - Pulmonary stenosis; SND - Sensorineural deafness; VSD - Ventricular septal defect.
listed chronologically by age of patient at death. No ear history was available in Cases 1 to 7. Case 8 had complained of total deafness of the left ear, which was confirmed at the age of nine years and which was suspected to be due to having had mumps or measles in early childhood. Case 9 had bilateral sensorineural deafness, restricted to 8 kHz, at 26 years of age. Case 10 had had right otitis media with a moderate conductive hearing loss which was followed by bilateral total deafness, felt to be secondary to the administration of an ototoxic drug (gentamycin) during an episode of sepsis which occurred two months prior to his death at age 35. HISTOPATHOLOGIC FINDINGS
The major pathological findings in 20 temporal bones (10 cases) are tabulated in Table 2. The numbers in parentheses are those of Figures 1-6 in this
article which demonstrate the pathology discussed. DISCUSSION
Congenital anomalies of the temporal bone associated with cardiac anomalies have been reported in numerous congenital diseases, such as genetic disorders.>" chromosomal abnormalltles.s-" and diseases associated with environmental factors. 14 ,1 s However, to our knowledge the occurrence of temporal bone anomalies in cases of congenital heart diseases in which the etiology is still unknown, have been substantiated only by Kelemen." He studied such cases as part of an aural anomaly series which involved description of various kinds of diseases, mostlv those which are seen in neonates. The findings in our study, therefore, may provide clinically worthwhile information which will extend the scope of his study since
Downloaded from aor.sagepub.com at Kungl Tekniska Hogskolan / Royal Institute of Technology on July 23, 2015
Downloaded from aor.sagepub.com at Kungl Tekniska Hogskolan / Royal Institute of Technology on July 23, 2015
-
- - - -
-
- - - - -
+ + + +
2
L
-
-
-
-
-
+ + + + + +(1) -
+ +
R
3
-
-
-
5
-
-
- -
-
-
-
+ -
-
+ -
-
- - -
- -
R L
- - - - -
4
R L
+
-
+(3) + +(2) +
-
L
+(2) +
-
R
- -
- - - -
- - - - - ++
- - - - - - -
7
R L
- - - - - - -
6
R L
+(4) +
+
- - - -
-
-
+
- - - -
-
- -
8
R L
(Numbers in parentheses indicate figures accompanying this article which illustrate the anomaly described.) • Anomaly of the structure closely related to the ectoderm.
Middle ear anomalies Mesenchymal tissue remnant Wide angle of facial genu" Stapedial artery persistence Large defect of facial canal High jugular bulb Ossicular anomaly Inner ear anomalies Shortened cochlea ° Anomaly of lateral canal" Anomaly of posterior canal" Cochlear aqueduct obliteration Patent utricular valve"
Case 1 Side R L
TABLE 2. PATHOLOGICAL FINDINGS
-
-
- - -
+ +
-
+ +
- - - -
-
- -
9
R L
-
+
- - -
+
+ + + +(6)
-
- -
- -
+ + - +(5) + +
10
R L
5 3 2 2 1
8 6 5 4 4 2
No. of Anomalies
TEMPORAL BONE ANOMALIES & HEART DISEASE
75
Fig. 1. (Case 2, R) High jugular bulb (large arrows), accompanied by dehiscence (small arrow) of the medial bony wall of the tympanic cavity. (H & E, X 11)
Fig. 3. (Case 3, R) The persistent stapedial artery (arrow) is seen. SF Stapes footplate. (H & E, X 52)
all of the cases in our study were older than one year (1-35 years). Our study is essentially an expanded complement to that of Kelemen."
Fig. 2. (Case 3, R) Wide defect of the facial canal (arrow) at its horizontal portion, remnant of mesenchymal tissue in the middle ear space, and circumscribed ossification of the posterior semicircular canal (P) are seen. (H & E, X 10.5)
The anomalies observed in the middle ears in this study were: mesenchymal tissue remnant (8 ears), wide angle of the facial genu (6 ears), stapedial artery persistence (5 ears), large defect of the facial canal in the horizontal portion (4 ears), a high jugular bulb (4 ears), and bulky incus (2 ears). Case 10 (L) exhibited several of these middle ear anomalies (Table 2).
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76
EGAMI ET AL
Fig. 4. (Case 8, L) Absence of the cochlear nerve to the basal turn is observed (arrows) in poorly developed modiolus. (H & E, X 27)
Fig. 5. (Case 10, R) The wide angle of the facial genu (arrow) is seen. C - Cochlea, L - Lateral crista. (H & E, X 17)
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TEMPORAL BONE ANOMALIES & HEART DISEASE
77
the cochlear aqueduct (2 ears), and a patent utriculoendolymphatic valve (1 ear). Case 10 (L) demonstrated the most severe anomalies, including not only an outpouching lateral canal (Fig. 6) but also a patent utriculoendolymphatic valve. The development of human inner ear structures which relate to these findings is as follows. At the fetal age of 10-11 weeks, two and one-half to 2:Ji turns of the cochlea are completed; the cochlear aqueduct attains its maximum size of 250ft at 20 fetal weeks and becomes narrower at a later time. In the vestibular system, at 8-9 weeks of fetal life, the utricular and saccular ducts are formed and the utriculoendolymphatic valve is seen; the superior canal, posterior canal, and lateral canal reach their maximum sizes at 20, 21, and 23 weeks of fetal life, respectively.l"-"
Fig. 6. (Case 10, L) Anomaly of the vestibular system with simple outpouching of the lateral semicircular canal (L) resulting in a wide vestibule (V). Posterior semicircular canal (P) is normal. (H & E, X 17)
The development of human middle ear structures which relate to the findings observed in our cases is as follows. At nine weeks of fetal age, the facial genu angle decreases and becomes almost a right angle; in the 9th to the 12th week the stapedial artery disappears; at 26 weeks, the tympanic portion of the facial canal begins to form from the laterohyale of Reichert's cartilage; at 36 weeks, the pneumatization of the tympanic cavity is completed; by 38 weeks the stapes, malleus, and incus have assumed an adult form; and around the time of birth the facial canal is found to be fully formed.v'-" The inner ear anomalies which were observed in this study consisted of a shortened cochlea (length less than 2)~ turns) (5 ears), anomalies of the lateral canal (3 ears), anomalies of the posterior canal (2 ears), obliteration of
Generalizations may be made regarding the occurrence of particular anomalies in temporal bones. 1) Most of the anomalies we observed in the middle and inner ears are due to arrested development and present features similar to those of structures found in various stages of fetal life. 2) Middle ear anomalies are more frequently observed (11 ears, 55%) that anomalies of the inner ear (7 ears, 35%). 3) Anomalies of the structures derived from the mesoderm are seen more frequently (13 ears, 65%) than those closely related to the ectoderm (9 ears, 45%) (Table 2). The authors were not certain as to the classification of the high jugular bulb as a congenital anomaly because of the lack of literature relating this finding to a developmental anomaly. Overton and Hitter!" reviewed sections of 257 individuals' temporal bones and found that the jugular bulb extended into the middle ear above the inferior rim of the bony annulus in 6% of the specimens. Our observation that a high jugular bulb is found more frequently (in 20% of our patients) in congenital heart patients than in the general population of Overton and Ritter's study-" may lead one to interpret this as a congenital anomaly.
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78
EGAMI ET AL
A definite relationship between the anomalies we have described and the hearing problems of these patients could not be established due to the multiplicity of the factors involved. However, observation of these anomalies, particularly of those observed in the middle ear (wide defect of the facial canal, high jugular bulb, and stapedial artery persistence) is of particular value as they may often be encountered during surgery.
CONCLUSION
This study of the temporal bones of congenital heart disease cases revealed that middle and inner ear abnormalities may coexist with anomalies of the heart. These ear abnormalities seemed to be due to an arresting of development, at various stages of fetal life, of mesodermal and ectodermal structures. These middle ear anomalies are significant from the surgical point of view, and thus are worthy of note.
REFERENCES 1. Konigsmark BW, Gorlin RJ: Genetic 10. Sando I, Leiberman A, Bergstrom L, et and Metabolic Deafness. Philadelphia, PA, W. al: Temporal bone histopathological findings B. Saunders Co, 1976 in trisomy 13 syndrome. Ann Otol Rhinol Lar2. Kelemen G: Aural participation in con- yngol 84( Suppl 21) 1975 genital malformations of the organism. Acta 11. Sando I, Bergstrom L, Wood RP, et al: Otolaryngol (Stockh) [Suppl 321] 1974 Temporal bone findings in trisomy 18 syndrome. Arch Otolaryngol 91 :552-559, 1970 3. Forney WR, Robinson SJ, Pascoe DJ: Congenital heart disease, deafness, skeletal mal12. Igarashi M, Takahasi M, Alford BR, et formation - a new syndrome? J Pediatr 68: al: Inner ear morphology in Down's syndrome. 14-26, 1966 Acta Otolaryngol (Stockh) 83:175-181, 1977 4. Lewis SM, Sonnenblick BP, Gilbert L, 13. Anderson H, Filipson R, Flurr E, et al: et al: Familial pulmonary stenosis and deafHearing impairment in Turner's syndrome. mutism. Am Heart J 55:458-462, 1958 Acta Otolaryngol (Stockh) [Suppl 247] 1969 14. Lindsay J, Caruthers D, Hemenway W, 5. Kelemen G: Hurler's syndrome and the et al: Inner ear pathology following maternal hearing organ. J Laryngol Otol 80:791-803, 1966 rubella. Ann Otol Rhinol Laryngol 62:12011218, 1953 6. Friedmann I, Fraser GR, Frogatt P: 15. Jorgensen M, Kristensen H, Buch N: Pathology of the ear in the cardio-auditory Thalidomide-induced aplasia of the inner ear. syndrome of [ervell and Lange-Nielsen. J LarJ Laryngol Otol 78:1095-1106, 1964 yngol Otol 80:451-470, 1966 16. Sando I, Izumi S: Congenital middle 7. Kelemen G: Marfan's syndrome and ear anomaly. Otologia (Fukuoka) 20:1-11, hearing organ. Acta Otolaryngol (Stockh) 59: 1974 23-32,1965 17. Bast H, Anson BJ: Temporal Bone and 8. Bretlau P, Jorgensen MB: Otosclerosis the Ear. Springfield, Ill, C. C. Thomas Co, and osteogenesis imperfecta. Acta Otolaryngol 1949 (Stockh) 67:269-276, 1969 18. Overton SB, Ritter FN: A high-placed 9. Bergstrom L, Stewart J, Kenyon B: Ex- jugular bulb in the middle ear: A clinical and ternal auditory atresia and the deleted chro- temporal bone study. Laryngoscope, 83: 19861991, 1973 mosome. Laryngoscope 84:1905-1917,1974 ACKNOWLEDGEMENTS - The authors are grateful to Mrs. Pamela Marks, Miss Shelly Frisch, Mrs. Cheryl Mervis, and Miss Jean MacPherson for their kind assistance in this study. REPRINTS - Isamu Sando, MD, DMS, Department of Otolaryngology, University of Pittsburgh School of Medicine, 230 Lothrop Street, Pittsburgh, PA 15213.
XII WORLD CONGRESS OF OTORHINOLARYNGOLOGY The XII World Congress of Otorhinolaryngology will be held June 21-26, 1981 in Budapest, Hungary. Preliminary communications, recommendations and suggestions, should be directed to Prof. Dr. L. Surj{m, President, XII World Congress of ORL, H-1389 Budapest, Pf: 112 Hungary.
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TEMPORAL BONE ANOMALIES ASSOCIATED WITH CONGENITAL HEART DISEASE TETSUYA EGAMI, MD, DMS ISAMU SANDO, MD, DMS
EUGENE N. MyERS, MD, FACS
PITTSBURGH, PENNSYLVANIA
A temporal bone histopathological study was conducted to detect temporal bone anomalies in 20 cars (10 cases) of individuals with congenital heart anomalies. We restricted our study to patients more than one year of age, and to heart anomalies of unknown etiology. The temporal bones were obtained from refrigerated cadavers, fixed in formalin, embedded in celloidin, cut in a horizontal plane, stained with H & E, and mounted on glass slides for light microscopic study. Anomalies observed in the middle ear were: remnants of mesenchymal tissue (8 ears), wide angle of the facial genu (6 cars), persistence of the stapedial artery (5 ears), large defect of the facial canal (4 ears), high jugular bulb (4 ears), and bulky incus (2 cars). Inner ear anomalies consisted of a shortened cochlea (5 ears), anomaly of the horizontal canal (3 ears), anomaly of the posterior canal (2 ears), obliteration of the cochlear aqueduct (2 ears), and patent utriculoendolymphatic valve (1 ear). Most of the anomalies observed appeared to be due to arrested development, and resembled features which may be found at various stages of fetal life. Structural anomalies were more commonly found in the mesoderm than in the ectoderm, and middle ear anomalies were more frequently encountered than anomalies of the inner ear. No definite relationship between these anomalies observed in the temporal bone and hearing problems which had been recorded clinically for these patients could be detected. However, the large defect (more than one third of the circumference) of the facial canal, the high jugular bulb, and the stapedial artery persistence should be recognized as problems since they may be encountered during middle ear surgery.
Congenital anomalies of the temporal findings of temporal bone histopathobone have been reported to occur in logic studies of such patients who lived association with other anomalies.' For longer than one year and, where posinstance, Kelemen- reported on the oc- sible, to emphasize the clinical sigcurrence of anomalies of the temporal nificance of these findings. bone in neonates and suggested that MATERIALS AND METHODS ear anomalies may coexist with congenital heart disease in some patients. This study made use of 20 temporal bones However, anomalies of the ear associ- from ten cases. The cases included only patients of more than one year of age whose maated with congenital heart disease, but jor significant problem was the presence of not associated with any known syn- cardiac anomalies. The temporal bones were drome or disease, have not been suf- removed from refrigerated cadavers, fixed in formalin, decalcified, embedded in celloidin, ficiently investigated. Recent developments in cardiac surgery have given congenital heart disease patients a prolonged life span. As a result, more associated ear problems may be encountered in these individuals than previously, including ear anomalies which require surgical intervention. The purpose of this paper is to report the
and sectioned horizontally at 20.u thickness. Every tenth section was stained with H & E and mounted on a glass slide for light microscopic study. CASE HISTORIES
Summaries of the ear histories, of the heart abnormalities, and of associated general anomalies which pertain to these cases are tabulated in Table 1,
From the Departments of Otolaryngology and Pathology, University of Pittsburgh School of Medicine, Eye and Ear Hospital. Pittsburgh, Pennsylvania; and the Department of Otolaryngology, University of COlorado Medical Center. Denver. Colorado. This study was supported In part by Research Grants NS 09293-05, NS 13787-01, and NS 12748-02 from the National Institute of Neurological and Communicable Diseases and Stroke.
12
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TEMPORAL BONE ANOMALIES & HEART DISEASE
73
TABLE 1. SUMMARIES OF EAR HISTORIES, CARDIAC ABNORMALITIES AND ASSOCIATED GENERAL ANOMALIES IN TEN CASES Case
Age
Sex
Ear History
Associated Abnormalities
Cardiac Abnonnality
1
12 mo
M
None
2
22 mo
M
None
3
3 yr
F
None
4
9yr
F
None
5
12 yr
F
None
6
13 yr
F
None
7
17 yr
M
None
8
20 yr
M
Lt. profound deafness
VSD, PS, surgery at age 8 yr
9
26 yr
F
VSD,PDA
10
35 yr
M
Bilateral SND (60 dB at 8 kHz) OMC with conductive deafness followed by Jrofound deafness ter ototoxic drug administration
ASD, died after cardiac surgery Transposition of great vessels, VSD Tetralogy of FaIlot, died after cardiac surgery A-V canal, died after cardiac surgery Tetralogy of FaIlot, surgery at age 5 yr A-V canal, transposition of great vessels ASD, VSD, PS, A-V canal, anomalous pulmcnary venous return
Tetralogy of FaIlot, surgery at age 12, died after second cardiac operation
Cleft soft palate None Duodenal atresia, Scleral dermoid Meckel's diverticulum None None Absence of spleen, supernumerary pulmonary lobulation Absence of right kidney and right testis Scoliosis and lordosis Periventricular brain cyst
ASD - Atrial septal defect; A-V canal - Atria-ventricular canal: OMC - Otitis media chronlea; PDA - Patent ductus arteriosus; PS - Pulmonary stenosis; SND - Sensorineural deafness; VSD - Ventricular septal defect.
listed chronologically by age of patient at death. No ear history was available in Cases 1 to 7. Case 8 had complained of total deafness of the left ear, which was confirmed at the age of nine years and which was suspected to be due to having had mumps or measles in early childhood. Case 9 had bilateral sensorineural deafness, restricted to 8 kHz, at 26 years of age. Case 10 had had right otitis media with a moderate conductive hearing loss which was followed by bilateral total deafness, felt to be secondary to the administration of an ototoxic drug (gentamycin) during an episode of sepsis which occurred two months prior to his death at age 35. HISTOPATHOLOGIC FINDINGS
The major pathological findings in 20 temporal bones (10 cases) are tabulated in Table 2. The numbers in parentheses are those of Figures 1-6 in this
article which demonstrate the pathology discussed. DISCUSSION
Congenital anomalies of the temporal bone associated with cardiac anomalies have been reported in numerous congenital diseases, such as genetic disorders.>" chromosomal abnormalltles.s-" and diseases associated with environmental factors. 14 ,1 s However, to our knowledge the occurrence of temporal bone anomalies in cases of congenital heart diseases in which the etiology is still unknown, have been substantiated only by Kelemen." He studied such cases as part of an aural anomaly series which involved description of various kinds of diseases, mostlv those which are seen in neonates. The findings in our study, therefore, may provide clinically worthwhile information which will extend the scope of his study since
Downloaded from aor.sagepub.com at Kungl Tekniska Hogskolan / Royal Institute of Technology on July 23, 2015
Downloaded from aor.sagepub.com at Kungl Tekniska Hogskolan / Royal Institute of Technology on July 23, 2015
-
- - - -
-
- - - - -
+ + + +
2
L
-
-
-
-
-
+ + + + + +(1) -
+ +
R
3
-
-
-
5
-
-
- -
-
-
-
+ -
-
+ -
-
- - -
- -
R L
- - - - -
4
R L
+
-
+(3) + +(2) +
-
L
+(2) +
-
R
- -
- - - -
- - - - - ++
- - - - - - -
7
R L
- - - - - - -
6
R L
+(4) +
+
- - - -
-
-
+
- - - -
-
- -
8
R L
(Numbers in parentheses indicate figures accompanying this article which illustrate the anomaly described.) • Anomaly of the structure closely related to the ectoderm.
Middle ear anomalies Mesenchymal tissue remnant Wide angle of facial genu" Stapedial artery persistence Large defect of facial canal High jugular bulb Ossicular anomaly Inner ear anomalies Shortened cochlea ° Anomaly of lateral canal" Anomaly of posterior canal" Cochlear aqueduct obliteration Patent utricular valve"
Case 1 Side R L
TABLE 2. PATHOLOGICAL FINDINGS
-
-
- - -
+ +
-
+ +
- - - -
-
- -
9
R L
-
+
- - -
+
+ + + +(6)
-
- -
- -
+ + - +(5) + +
10
R L
5 3 2 2 1
8 6 5 4 4 2
No. of Anomalies
TEMPORAL BONE ANOMALIES & HEART DISEASE
75
Fig. 1. (Case 2, R) High jugular bulb (large arrows), accompanied by dehiscence (small arrow) of the medial bony wall of the tympanic cavity. (H & E, X 11)
Fig. 3. (Case 3, R) The persistent stapedial artery (arrow) is seen. SF Stapes footplate. (H & E, X 52)
all of the cases in our study were older than one year (1-35 years). Our study is essentially an expanded complement to that of Kelemen."
Fig. 2. (Case 3, R) Wide defect of the facial canal (arrow) at its horizontal portion, remnant of mesenchymal tissue in the middle ear space, and circumscribed ossification of the posterior semicircular canal (P) are seen. (H & E, X 10.5)
The anomalies observed in the middle ears in this study were: mesenchymal tissue remnant (8 ears), wide angle of the facial genu (6 ears), stapedial artery persistence (5 ears), large defect of the facial canal in the horizontal portion (4 ears), a high jugular bulb (4 ears), and bulky incus (2 ears). Case 10 (L) exhibited several of these middle ear anomalies (Table 2).
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76
EGAMI ET AL
Fig. 4. (Case 8, L) Absence of the cochlear nerve to the basal turn is observed (arrows) in poorly developed modiolus. (H & E, X 27)
Fig. 5. (Case 10, R) The wide angle of the facial genu (arrow) is seen. C - Cochlea, L - Lateral crista. (H & E, X 17)
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TEMPORAL BONE ANOMALIES & HEART DISEASE
77
the cochlear aqueduct (2 ears), and a patent utriculoendolymphatic valve (1 ear). Case 10 (L) demonstrated the most severe anomalies, including not only an outpouching lateral canal (Fig. 6) but also a patent utriculoendolymphatic valve. The development of human inner ear structures which relate to these findings is as follows. At the fetal age of 10-11 weeks, two and one-half to 2:Ji turns of the cochlea are completed; the cochlear aqueduct attains its maximum size of 250ft at 20 fetal weeks and becomes narrower at a later time. In the vestibular system, at 8-9 weeks of fetal life, the utricular and saccular ducts are formed and the utriculoendolymphatic valve is seen; the superior canal, posterior canal, and lateral canal reach their maximum sizes at 20, 21, and 23 weeks of fetal life, respectively.l"-"
Fig. 6. (Case 10, L) Anomaly of the vestibular system with simple outpouching of the lateral semicircular canal (L) resulting in a wide vestibule (V). Posterior semicircular canal (P) is normal. (H & E, X 17)
The development of human middle ear structures which relate to the findings observed in our cases is as follows. At nine weeks of fetal age, the facial genu angle decreases and becomes almost a right angle; in the 9th to the 12th week the stapedial artery disappears; at 26 weeks, the tympanic portion of the facial canal begins to form from the laterohyale of Reichert's cartilage; at 36 weeks, the pneumatization of the tympanic cavity is completed; by 38 weeks the stapes, malleus, and incus have assumed an adult form; and around the time of birth the facial canal is found to be fully formed.v'-" The inner ear anomalies which were observed in this study consisted of a shortened cochlea (length less than 2)~ turns) (5 ears), anomalies of the lateral canal (3 ears), anomalies of the posterior canal (2 ears), obliteration of
Generalizations may be made regarding the occurrence of particular anomalies in temporal bones. 1) Most of the anomalies we observed in the middle and inner ears are due to arrested development and present features similar to those of structures found in various stages of fetal life. 2) Middle ear anomalies are more frequently observed (11 ears, 55%) that anomalies of the inner ear (7 ears, 35%). 3) Anomalies of the structures derived from the mesoderm are seen more frequently (13 ears, 65%) than those closely related to the ectoderm (9 ears, 45%) (Table 2). The authors were not certain as to the classification of the high jugular bulb as a congenital anomaly because of the lack of literature relating this finding to a developmental anomaly. Overton and Hitter!" reviewed sections of 257 individuals' temporal bones and found that the jugular bulb extended into the middle ear above the inferior rim of the bony annulus in 6% of the specimens. Our observation that a high jugular bulb is found more frequently (in 20% of our patients) in congenital heart patients than in the general population of Overton and Ritter's study-" may lead one to interpret this as a congenital anomaly.
Downloaded from aor.sagepub.com at Kungl Tekniska Hogskolan / Royal Institute of Technology on July 23, 2015
78
EGAMI ET AL
A definite relationship between the anomalies we have described and the hearing problems of these patients could not be established due to the multiplicity of the factors involved. However, observation of these anomalies, particularly of those observed in the middle ear (wide defect of the facial canal, high jugular bulb, and stapedial artery persistence) is of particular value as they may often be encountered during surgery.
CONCLUSION
This study of the temporal bones of congenital heart disease cases revealed that middle and inner ear abnormalities may coexist with anomalies of the heart. These ear abnormalities seemed to be due to an arresting of development, at various stages of fetal life, of mesodermal and ectodermal structures. These middle ear anomalies are significant from the surgical point of view, and thus are worthy of note.
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XII WORLD CONGRESS OF OTORHINOLARYNGOLOGY The XII World Congress of Otorhinolaryngology will be held June 21-26, 1981 in Budapest, Hungary. Preliminary communications, recommendations and suggestions, should be directed to Prof. Dr. L. Surj{m, President, XII World Congress of ORL, H-1389 Budapest, Pf: 112 Hungary.
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