X-linked Agammaglobulinemia Diagnosed Following Bezold's Abscess: A Case Report.

Bezold's abscess is an extracranial complication of otitis media, in which a cervical abscess forms from the mastoid process through an ostial fistula, and is a rare condition in recent years. In this study, we experienced a X-linked agammaglobulinemia, which was discovered due to Bezold's abscess. Case: A 12-year-old boy suffering from recurrent right suppurative otitis media for three months was treated with tympanostomy and oral antibacterial therapy at a local otorhinolaryngology clinic. The patient visited the clinic due to a recurrence of symptoms. CT showed bony defects in the cortical bone and mastoid process of the lateral side of the right mastoid cell. The patient was referred to our hospital, admitted the same day and underwent emergency surgery. Intraoperative findings led to the diagnosis of acute mastoiditis and Bezold's abscess c aused b y mastoiditis spreading to the s ternocleidomastoid muscle. After drainage and administration of ABPC/SBT, the abscess disappeared, and the patient's general condition improved. Subsequently, a blood typing test performed on admission suggested the influence of low immunoglobulin levels. A close examination by the pediatric department led to a diagnosis of X-linked agammaglobulinemia. As a result, the patient receives regular immunoglobulin therapy and has been free of infection, including Bezold's abscess. CONCLUSIONS: In the case of recurrent otitis media and rare infections, congenital immune abnormalities should be considered.

A Case of Intratemporal Rhabdomyosarcoma in a Child Presenting with VIIth, IXth, and Xth Cranial Nerve Paralysis.

OBJECTIVE: Rhabdomyosarcoma is the most common soft tissue tumor in children, with average age of onset being 5 years, and approximately 70% cases diagnosed below 10 years of age. It accounts for 37% of primary head and neck malignancies in children. Chemotherapy with surgery, and radiation is selected as the primary treatment. We report a rare case of rhabdomyosarcoma in the temporal bone presenting with glossopharyngeal and vagus nerve paralysis as well as facial palsy. CASE REPORT: The patient was a 6-year-old boy, and his initial symptom was dizziness followed by facial palsy and hoarseness. Although a severe type of otitis media was suspected in the first clinic, CT and MRI showed a temporal bone tumor with parameningeal extension. Biopsy with cortical mastoidectomy revealed an embryonal-type rhabdomyosarcoma. Pretreatment re-excision was abandoned because of parameningeal involvement. The tumor disappeared after a series of chemotherapy, however, meningeal dissemination occurred, and he eventually died even after an additional administration of anti-cancer agents and intensive modulated radiation therapy. CONCLUSION: In the case of facial palsy concomitant with other cranial nerve paralysis, care must be taken into neoplastic origin. Early image diagnosis may offer a chance of complete resection in addition to chemoradiotherapy.

Cholesterol granuloma presenting with facial nerve paralysis following translabyrinthine surgery: A case report.

BACKGROUND: Cholesterol granuloma (CG) is a foreign body giant cell inflammatory reaction to blood degradation products, and clinically presents as a cyst with a thick fibrous lining filled with brownish-yellow fluid. We report an unusual case of CG with facial nerve paralysis 9 years after translabyrinthine (TL) surgery for vestibular schwannoma (VS) removal. CASE: A 70-year-old woman, who underwent TL surgery previously, presented with left facial palsy (FP). CT findings revealed devastated cochlea and uncovered facial nerve, and progression of FP up to House-Brackmann grade 6 was observed. Therefore, we suspected recurrence of cystic VS or facial schwannoma, although MRI was inconsistent with that of schwannoma. The cyst was completely surgically excised, and histopathology confirmed CG diagnosis. Postoperatively her facial movement improved to grade 4. Recurrence has not been encountered since 7 years. CONCLUSION: During follow-up after TL surgery for VS, care should be taken for possibility of CG occurring.

Concurrent chemoradiotherapy with docetaxel for T2 laryngeal carcinoma.

OBJECTIVE: To evaluate the efficacy and safety of concurrent chemoradiotherapy with docetaxel (DOC) for T2 laryngeal carcinoma. METHOD: Twelve patients with T2 laryngeal cancer received concurrent chemoradiotherapy (60-70Gy) with weekly DOC (10 mg/m2) (CCRT group). The clinical files of the patients were retrospectively reviewed and survival rates and laryngeal preservation rates were analyzed compared with the group treated with conventional radiation therapy alone (RT group). RESULTS: Complete response was observed in 11 of 12 patients (91.7%) in the CCRT group. Overall 5-year survival rates by Kaplan-Meier's method were 90% for the CCRT group and 78% for the RT group. The disease free survival with anterior commisure involvement was 90% in CCRT group and 53% in RT group, respectively. Toxicity over grade III was noticed in 3 patients. CONCLUSION: Concurrent chemoradiotherapy with DOC is a feasible and effective treatment modality for organ preservation in T2 laryngeal cancer in the outpatient setting. Efficacy and safety of this treatment modality in an outpatient setting were discussed.

Personal factors involved in onset or progression of Ménière's disease and low-tone sensorineural hearing loss.

PURPOSE: To identify personal causative factors for Ménière's disease. PROCEDURES: Patterns of hearing loss progression were studied in patients with Ménière's disease and low-tone sensorineural hearing loss, and the involvement of stress and the relation of stressors to the onset or progression of the disease were analyzed. RESULTS: Low-tone loss recurred in 40% of patients even after hearing was restored, and low-tone loss progressed to high-tone loss after frequent repetitions of recovery and recurrence. High-tone loss tended to proceed to all-tone loss. Eighty percent of patients reported that stress was involved or deeply involved in the onset or progression of the disease. Common causative stressors were business-related pressure, insufficient sleep, and troubles at the workplace or at home. CONCLUSIONS: The present findings indicate that recovery and recurrence may be influenced by the strength and duration of stress that is produced when patients do not feel rewarded for engrossment in their work or for self-inhibiting behaviors.

Comparative study of the daily lifestyle of patients with Meniere's disease and controls.

OBJECTIVES: This study was performed to investigate the possibility that daily lifestyle may have a causal relationship with Meniere's disease. METHODS: We conducted a questionnaire study of daily lifestyles among groups of patients with Meniere's disease and those with low-frequency hearing loss, and compared the results with those of control groups of local residents matched individually by gender and age. RESULTS: The Meniere's disease group diverged most widely from the control groups in their behavior patterns. Significant divergence was especially indicated in their engrossed, self-inhibiting, and time-constrained behaviors. Although the low-frequency hearing loss group also exhibited similar tendencies toward engrossment and in their feeling pressed for time, their self-inhibiting behavior was less pronounced. There was no major difference between the endolymphatic hydrops patient groups and the control groups on other items in the study such as daily lifestyle, environmental stress, and means of relaxation. CONCLUSIONS: The results of the present study strongly suggest that there may be a link between an individual's specific behavior patterns and the onset of Meniere's disease.

A case of accessory parotid gland tumor.

We herein describe a case of accessory parotid gland tumor, including diagnosis and surgical approach. The accessory parotid gland is salivary tissue separated from the main parotid gland and lying on masseter muscle. It has secondary duct empting into the Stensen's duct. The accessory parotid gland exists in 21-61 % of individuals. However, the appearance of an accessory parotid tumor is rare, with a reported frequency of 1-7.7 % of all parotid gland tumors. Surgical resection is the treatment of choice for the accessory parotid gland tumor. It is important to identify the buccal branch of the facial nerve to avoid injury to the facial nerve. The tumor in our case was surgically resected without facial nerve injury. The histopathological diagnosis was pleomorphic adenoma.

[Two patients with distant metastases from head and neck squamous cell carcinomas successfully treated with docetaxel and cisplatin].

Two cases of distant metastases from head and neck squamous cell carcinomas are reported. The patients were treated intravenously with docetaxel (60 mg/m2) in combination with cisplatin (70 mg/m2). The chemotherapy was repeated every three weeks. Both cases showed a remarkable response. A complete response was obtained in one case. Grade 1 nausea and alopecia were observed. One patient had grade 3 neutropenia, which was treated with G-CSF. We herein describe the efficacy of newly developed docetaxel with cisplatin in treating distant metastasis from head and neck cancer.