Klippel-Feil Syndrome with Bilateral Congenital Aural Atresia and Bilateral Congenital Deafness / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: The Klippel-Feil syndrome is a congenital anomaly characterized by fusion of the cervical vertebrae. It is often associated with serious congenital anomalies of the nervous, cardivascular, respiratory, and urogenital systems. Another anomaly often associated is hearing loss. For those cases of the Klippel-Feil syndrome accompanying hearing loss, the middle and inner ear deformities were reported in some, whereas aural atresia was extremely rare. The purpose of this paper is to report the fact that Klippel-Feil syndrome can be combined with congenital aural atresia and deafness. MATERIAL & METHOD: Recently we experienced a case of Klippel-Feil syndrome in a 28 year old female. In this case, the second and third cervical vertebrae were fused. The patient also presented a short neck, cardiovascular anomaly, bilateral congenital deafness, and bilateral congenital aural atresia. Radiographical observation showed soft tissue density in the bilateral external uditory canals and stenosis of bilateral internal auditory canals, but no definite middle and inner ear anomalies in the temporal bone were found by computerized tomogram. RESULT: As operative procedures, canaloplasty and meatoplasty were performed after cholesteatoma removal from the external auditory canal. The external auditory canal has been well maintained following the surgery. CONCLUSION: For patients with the Klippel-Feil syndrome, we must do full evaluation of the whole body in order to treat first any life threatening problems. Evaluation of hearing should then be followed to implement appropriate treatment and early rehabilitation.

Vascularized Fascia as a Transferable Bed for Experimental Laryngeal Reconstruction / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: Free fascia flaps provide neovascularity without adding bulk and are particularly suited to serve in microvascular transfers to recipient beds carrying an epithelial lining. As such, free fascia flaps may be useful in providing a mucosal lining at the interior side of the reconstructed larynx. This study examines the reliability of a composite reconstruction method using a combined vascular flap for the laryngeal defects. MATERIALS AND METHODS: A vascularized fascial flap which was consistently present and easy to isolate was developed in dogs. Laryngeal defects were reconstructed with a combined vascular flap consisting of lateral thoracic fascia, which is also used as a transferable vascular be). A buccal mucosa graft was fixed on this bed to provide as an internal lining. An autogenous auricular cartilage graft, circumferentially wrapped in vascular connective tissue, was used as an external support. RESULTS: There were no respiratory distress, limitation of activity, and laryngeal stenosis after the laryngeal reconstruction. CONCLUSION: We have maintained the positive aspects as well as improved the negative aspects of the experiment and devised a new series of experiments which are now in progress. The lateral thoracic fascia is used as a transferable bed with an autogenous cartilage for support. They may be a useful tool for laryngotracheal reconstruction.

A Clinical Study of Facial Nerve Paralysis / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: Since Sir Charles Bell (1829) demonstrated that motor innervation of the muscles of facial expression is by cranial nerve VII, many more research have contributed to the diagnosis and treatment of facial nerve paralysis. We attempted to evaluate the causes, arrive at accurate diagnosis and determine the treatment for facial nerve paralysis. MATERIALS AND METHODS: The authors analyzed 65 cases of facial nerve paralysis diagnosed at the Department of Otolaryngology, Hanyang University Hospital from January 1993 to December 1995. RESULTS: 1) The most common age group was the twenties. 2) The most common cause was trauma (23 cases, 35.4%). 3) The degree of facial nerve paralysis that were most common on first visits was Grade IV, based on the House-Brackmann grading method. 4) 38 cases (58.5%) were treated with chemotherapy and 27 cases (41.5%) were treated with surgical therapy. 5) Surgical techniques used were facial nerve decompression in 11 cases (40.8%), end to end anastomosis in 4 cases (14.8%), myringotomy with v-tube insertion in 4 cases (14.8%), tumor resection in 3 cases (11.1%), nerve graft in 2 cases (7.4%), mastoidectomy only in 2 cases (7.4%), and muscle transposition in one case (3.7%). CONCLUSION: In our review, the the most common degree of facial nerve paralysis on patients' first visits was Grade IV according to the House-Brackmann grading method. Statistically, there was no sexual difference in the incidences of facial nerve pals. The causes of facial nerve palsy due to trauma are recently on the rise because of increased traffic accident. However, iatrogenic incidents are decreasing because of the advance in the technical and instrumental development.