Repair of Spontaneous Cerebrospinal Fluid Otorrhea from Defect of Middle Cranial Fossa

Spontaneous cerebrospinal fluid (CSF) otorrhea is defined as CSF otorrhea where there are no identifiable causes including previous trauma, surgery, infection, neoplasm or congenital anomaly. The condition is rare. The origin of CSF leak is commonly a defect in the tegmen of the middle cranial fossa. The pathophysiology of spontaneous CSF otorrhea is unclear. Two theories of the etiology of bony defects of the temporal bone are the congenital bony defect theory and arachnoid granulation theory. The authors experienced a case of a 49-year-old female patient admitted with the complaint of persistent right ear fullness. Computed tomography revealed a large defect of the middle fossa and suspicious CSF otorrhea through the defect of tegmen tympani. Repair was successful with multiple bone chips using the transmastoid approach. The postoperative course was good and there has been no recurrence of the CSF leakage.

A Case of Solitary Neurofibroma of the External Auditory Canal / 대한이비인후과학회지

Neurofibroma is a relatively common neurogenic tumor originating from schwann cells or fibroblasts in peripheral nerve sheaths. It is classified as solitary or multiple. Solitary lesions are not usually associated with neurofibromatosis or von Recklinghausen's disease. We report a case of solitary neurofibroma occurred in the right external auditory canal (EAC) in a 30-year-old male who complained of progressive hearing loss and earfullness for 5 years. This diffuse painless mass displaced the auricle laterally and obstructed EAC completely. The lesion was partially excised by retroauricular approach and diagnosed as neurofibroma. This patient does not have any other evidences of neurofibromatosis. A complete excision in this case was not mandatory as malignant change is rare; moreover, it would require extensive resection of periauricular skin, subcutaneous tissue, cartilaginous ear canal and temporalis muscle, followed by reconstruction. We present the clinical characteristics and therapeutic methods of a solitary neurofibroma originating from EAC.

Reconstruction of the Posterior Canal Wall with Mastoid Obliteration after Canal Wall Down Mastoidectomy / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: There have been heated controversies over the choice of the canal wall down mastoidectomy (CWD) and canal wall up mastoidectomy (CWU), which are operational methods used to eliminate the lesion of cholesteatoma. Combining the advantages of both methods, we reconstructed the posterior canal wall with conchal cartilage plate and obliterated mastoid cavity with bone chips (group I), or hydroxyapatite mixed with bone chips (group II) since 2001. This study was designed to evaluate the surgical outcomes of posterior canal wall reconstruction with mastoid obliteration in the treatment of cholesteatoma. SUBJECTS AND METHOD: From January of 2001 to March of 2007, the posterior canal wall reconstruction with mastoid obliteration was conducted on 66 patients. There were 30 cases of cholesteatoma and 36 cases of old radical cavity. The postoperative observation period ranged from 5 to 74 months, with the average period of 34.7 months. We analyzed the postoperative complications, and hearing results of the 33 ossicular reconstruction cases. RESULTS: There was 1 case of residual cholesteatoma in the middle ear cavity, but no recurrent cholesteatoma. In most cases, reconstructed canal wall was maintained well, but partial canal wall resorption and postauricular dimpling occurred in 5 cases of group I. On the other hand, the epithelization of posterior canal wall was incomplete in 4 cases of group II. After surgery, no patients complained any cavity problems at all. CONCLUSION: The present study suggests that this procedure can prevent cavity problems and reduce the recurrence of cholesteatoma with destructed canal wall.

A Case of Type 2 Usher Syndrome / 대한이비인후과학회지

The Usher syndrome is an autosomal recessive disorder that cause bilateral sensorineural hearing loss and progressive loss of vision. It is genetically heterogeneous and is the most frequent cause of hereditary deafness and blindness in human. There are three types of Usher syndrome that can be distinguished clinically and into different subtypes. Type 2 Usher syndrome is the most common form and less severe than Type 1. It is characterized by congenital, moderate to severe, high frequency sloping hearing loss, retinitis pigmentosa which is typically diagnosed in late adolescence, and normal vestibular function. Recently, we have experienced a case of clinically diagnosed Type 2 Usher syndrome in a 34 years old female. We report this case with a brief review of literature. This is the first Type 2 Usher Syndrome to be reported in the otolaryngologic field in Korea.

Comparison of the Efficacy of Systemic and Intratympanic Steroid Treatment on Sudden Sensorineural Hearing Loss with Diabetes / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: High dose systemic steroid therapy is currently the mainstay of the treatment for sudden sensorineural hearing loss (SSNHL). However, it makes a glycemic control worse in patients with diabetes. Intratympanic steroid injection (ITSI) can result in reduced systemic steroid toxicity and higher perilymph steroid level selectively. The purpose of this study is to compare the efficacy of ITSI (IT group) with that of systemic steroid (IV group) on SSNHL with diabetes. SUBJECTS AND METHOD: Thirty eight SSNHL patients who were diagnosed with diabetes were divided into the IV group (19 patients) and the IT group (19 patients). In the IV group, prednisolone was administrated intravenously for 7 days followed by tapered doses orally for 7 days. In the IT group, dexamethasone was administrated 4 times within a 2 week-period. Hearing outcome was assessed before and after treatment. RESULTS: In the IV group, 10 patients (58.8%) showed an improvement in the pure tone audiogram (PTA), with a mean improvement of 17.6 dB (p=0.023). In the IT group, 16 patients (84.2%) showed improvement in the PTA, with the mean improvement of 25.1 dB (p=0.000). But there was no significant difference in hearing gain and the recovery rate between the two groups. And it is more difficult to control blood sugar in the IV group rather than in the IT group. CONCLUSION: ITSI treatment is as effective as the systemic steroid treatment for SSNHL patients with diabetes and it can avoid a significant side effect of systemic steroids. So it could be considered as an initial treatment for the SSNHL patient with diabetes.

A Case of Repair Using Hydroxyapatite Cement for Recurrent Perilymphatic Fistulas Occurred at Round Window After Cholesteatoma Surgery / 대한이비인후과학회지

Perilymphatic fistula (PLF) is a rare condition characterized by abnormal leakage of perilymph from the inner ear into the middle ear via a defect in the oval or round windows. PLF needs special attention concerning early diagnosis and treatment, because it can cause dizziness, hearing loss, tinnitus, meningitis, and other symptoms. The diagnosis of PLF usually cannot be confirmed before exploratory tympanotomy. Fascia, perichondrium, loose areolar tissue, fat and gelfoam can be used effectively in the PLF repair. Fibrin glue was used to aid in securing the graft material. However, recurrence is not rare and revision is sometimes needed to relieve the symptoms associated with the leakage of perilymph. So otologic surgeons should keep in mind that PLF has a great tendency to recur. Careful surgical procedures together with strict postoperative management are important to prevent recurrence and hydroxyapatite cement (HAC) can be used to cover the fistula area as a alternative graft material in recurrent perilymphatic fistula. We report on one recurrent case of postoperative PLF occurring at the round window that was repaired with HAC.

Results of Epitympanoplasty with Mastoid Obliteration Technique / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: There have been heated controversies over the choice of the canal wall down mastoidectomy (CWD) and canal wall up mastoidectomy (CWU), which are operational methods used to eliminate the lesion of chronic otitis media including cholesteatoma. The CWD method can secure a good operation field and remove the lesion easily, but it accompanies a cavity problem. The CWU method also has its shortcomings as it is difficult to eliminate cholesteatoma completely using this method. Combining the advantages of both methods, we invented a new operational method. The present study assesses the results of its use after a follow up of 55 months. MATERIALS AND METHODS: From December of 1994 to March of 1997, epitympanoplasty with mastoid obliteration was conducted on 44 adults (42 patients). Of these, 38 cases were cholesteatomas, 2 cases adhesive otitis media and 4 cases chronic otitis media with poor E-tube function. The postoperative observation period ranged from 41 to 68 months, with the average period of 55.2 months. RESULTS: There were 3 cases of residual cholesteatoma in the mastoid cavity and 3 cases in the middle ear cavity. All residual cholesteatomas in the mastoid cavity were treated with CWU, and there was no recurrent cholesteatoma or otorrhea, nor retraction pocket. CONCLUSION: Although the observation period of the postoperative process was not long enough, there was no recurrence. We suggest that it is advantageous to operate on cholesteatoma using the new method.

A Case of Usher Syndrome / 대한이비인후과학회지

The usher syndrome (US) is an autosomal recessive disorder characterized by congenital bilateral sensorineural hearing loss and progressive visual loss secondary to retinitis pigmentosa. It is the most common cause of the hereditary combined deafness-blindness in the western world. Three different types of US are recognized by clinical criteria. The US type I has severe to profound hearing loss, vestibular dysfunction, and prepubertally diagnosed retinitis pigmentosa, while the US type II has moderate to severe hearing loss, normal vestibular function, and later onset of retinitis pigmentosa. The US type III has a progressive hearing loss and retinitis pigmentosa with variable vestibular involvement. The diagnosis is confirmed by medical history and thorough otoscopical, audiologic, vestibular, and ophthalmological examinations. We have recently experienced a case of the US type I and report this with a brief review of the related literature.

The Rosai-Dorfman Disease of the Nasal Cavity and Salivary Gland: A Case Report / 대한이비인후과학회지

Rosai-Dorfman disease (RDD), or sinus histiocytosis with massive lymphadenopathy, is a rare idiopathic histiocytosis that most commonly involves the cervical lymph nodes, Extranodal involvement occurs in 30- 40% of cases, most often in the head and neck. Characteristic histological findings include emperiopolesis (intracellular engulfment) of lymphocytes and S-100 protein positivity. Treatment of Rosai-Dorfman disease is unnecessary unless the disorder becomes life-threatening, since the disease will resolve spontaneously in most patients. We experienced a case of extranodal RDD at the nasal cavity as the initial lesion prior its nodal involvement. The patient was a 18-year-old woman who complained of nasal obstruction for 4 years and had a submandibular mass that persisted during the last 3 months. Histologically, all specimens taken from the left nasal cavity, the left submandibular mass and the left upper jugular lymph node showed heavy infiltration of plasma cells, lymphocytes and sheets of macrophages with abundant pale cytoplasm which replaced the existing organ architecture.