Congenital Deformity of the Internal Auditory Meatus and Labyrinth

Author(s):  
Peter D. Phelps ◽  
Glyn A. S. Lloyd
2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Asiyeh Shojaee ◽  
Firooze Ronnasian ◽  
Mahdiyeh Behnam ◽  
Mansoor Salehi

AbstractBackgroundSirenomelia, also called mermaid syndrome, is a rare lethal multi-system congenital deformity with an incidence of one in 60,000–70,000 pregnancies. Sirenomelia is mainly characterized by the fusion of lower limbs and is widely associated with severe urogenital and gastrointestinal malformations. The presence of a single umbilical artery derived from the vitelline artery is the main anatomical feature distinguishing sirenomelia from caudal regression syndrome. First-trimester diagnosis of this disorder and induced abortion may be the safest medical option. In this report, two cases of sirenomelia that occurred in an white family will be discussed.Case presentationWe report two white cases of sirenomelia occurring in a 31-year-old multigravid pregnant woman. In the first pregnancy (18 weeks of gestation) abortion was performed, but in the third pregnancy (32 weeks) the stillborn baby was delivered by spontaneous vaginal birth. In the second and fourth pregnancies, however, she gave birth to normal babies. Three-dimensional ultrasound imaging showed fusion of the lower limbs. Neither she nor any member of her family had a history of diabetes. In terms of other risk factors, she had no history of exposure to teratogenic agents during her pregnancy. Also, her marriage was non-consanguineous.ConclusionThis report suggests the existence of a genetic background in this mother with a Mendelian inheritance pattern of 50% second-generation incidence in her offspring.


2008 ◽  
Vol 53 (No. 8) ◽  
pp. 452-455 ◽  
Author(s):  
J. Park ◽  
K.R. Cho ◽  
B.C. Sutradhar ◽  
D. Chang ◽  
S.H. Choi ◽  
...  

Congenitally deformed tibia-fibulas of both hind limbs were diagnosed in a four-month-old, female, domestic shorthair cat that was unable to bear weight and whose movement was painful on both stifle and hock joints. Bleeding was observed repeatedly from the wound made by deformed tibias at the cranial sides of hock joints where the bones were exposed. Radiography and computed tomography revealed a corn-shaped tibia and bow shaped fibula which extended cranio-distally without formation of the hock joints. Femoro-tarsal arthrodesis was successfully executed on both hind legs after exclusion of the deformed and pliable tibia-fibulas. Follow-up radiography showed that bone fusions had gradually improved and were without complications. Postoperatively, the cat was capable of walking on the corrected hind legs and running on the movement of hip joints. To the authors’ knowledge, this is the 1st reported case of femoro-tarsal arthrodesis in a cat. In this case, femoro-tarsal arthrodesis resulted in a satisfactory outcome for congenitally deformed tibia-fibulas in cat.


2006 ◽  
Vol 104 (4) ◽  
pp. 621-624 ◽  
Author(s):  
Han Soo Chang ◽  
Masahiro Joko ◽  
Joon Suk Song ◽  
Kiyoshi Ito ◽  
Tatsushi Inoue ◽  
...  

✓Extradural unroofing of the optic canal and subsequent mobilization of the optic nerve is a useful technique in the surgical treatment of parasellar tumors; however, the drilling procedure itself is associated with the risk of optic nerve damage. A safer technique would certainly be beneficial. The ultrasonic bone curette is a device developed in Japan for safer bone removal. Its use in intradural anterior clinoidectomy and opening of the internal auditory meatus has been reported before. In this article the authors describe their experience in using this device for extradural unroofing of the optic canal in patients with parasellar tumors. Between March 2002 and November 2004, the aforementioned technique was used in the treatment of eight patients with parasellar tumors. After undertaking a frontotemporal craniotomy and orbital osteotomy, an ultrasonic bone curette was used to unroof the optic canal via an epidural approach; in five cases anterior clinoidectomy was added subsequently. Using an ultrasonic bone curette, unroofing of the optic canal was completed safely and required much less expertise than that required for standard drilling. The mortality and major morbidity rates were 0%. The visual function outcome was satisfactory, with the overall visual status improving in all seven patients in whom this symptom was present preoperatively. The ultrasonic bone curette makes the unroofing of the optic canal safer and easier, possibly improving the visual outcome of patients undergoing surgery for parasellar tumors.


1976 ◽  
Vol 11 (3) ◽  
pp. 109-111 ◽  
Author(s):  
B. Azar-Kia ◽  
E. Palacios ◽  
M. Spak

2008 ◽  
Vol 123 (5) ◽  
pp. 563-565 ◽  
Author(s):  
A Jain ◽  
M Ablett ◽  
P Wardrop

AbstractObjectives:We report a very rare case of prostatic metastasis in the internal auditory meatus, which disappeared with treatment.Case report:An elderly man presented with a history of hearing loss, dizzy spells and, more recently, facial palsy. He also complained simultaneously of urological symptoms, which on investigation revealed advanced, metastatic prostate cancer. Radiological investigation, in the form of magnetic resonance imaging, revealed an internal auditory meatus mass which resembled an acoustic neuroma. The patient was treated with hormone injections.Tumours of the internal auditory meatus and cerebellopontine angle are mostly primary. Rarely, metastatic deposits have been described in this region, from squamous cell carcinoma, malignant melanoma, malignant parotid oncocytoma, renal carcinoma, and lung and thyroid primaries.Conclusion:To our knowledge, this is a very rare report in the world literature of prostatic metastasis to the internal auditory meatus. We discuss the common presenting features, investigations and treatment options for metastatic prostatic tumours of the internal auditory meatus and cerebellopontine angle.


BMJ ◽  
1960 ◽  
Vol 2 (5210) ◽  
pp. 1450-1451 ◽  
Author(s):  
D. Browne
Keyword(s):  

The Lancet ◽  
1918 ◽  
Vol 191 (4943) ◽  
pp. 738
Author(s):  
BarbaraG.R. Crawford

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