Different distribution of HLA class II alleles according to response to corticosteroid therapy in sudden sensorineural hearing loss.

OBJECTIVE: To investigate the association of HLA class II alleles with the susceptibility to sudden sensorineural hearing loss and with the results of corticosteroid treatment in the Korean population. DESIGN: HLA-DRB1, -DQA1, -DQB1, and -DPB1 genotyping by the sequence-specific oligonucleotide probes method in 41 patients with sudden sensorineural hearing loss and in 206 healthy control subjects. Initial hearing levels at the onset of hearing loss and final hearing levels after treatment were evaluated for the association with HLA class II alleles. SETTING: Tertiary care referral center, ambulatory and hospitalized care. SUBJECTS: Forty-one patients (24 men and 17 women; mean age, 49.2 years) were compared with 206 controls. Patients were divided into 2 groups according to their response to corticosteroid therapy (good response vs nonresponse). RESULTS: The frequencies of HLA-DRB1, -DQA1, -DQB1, and -DPB1 alleles were not significantly different between patients and controls (P>.05). When an association between the results of corticosteroid treatment and the frequency of HLA alleles was evaluated, the frequencies of HLA-DRB1*14 (relative risk [RR] = 3.5, P<.02), -DQA1*03 (RR = 4.2, P<.02), and -DQA1*05 (RR = 3.1, P<.03) were significantly increased, but HLA-DQA1*01 (RR = 0.2, P<.004) and -DQB1*06 (RR = 0.2, P<.009) were decreased in the group nonresponsive to corticosteroid therapy, compared with the controls. The distribution of HLA-DQA1*01 (P<.04), -DQB1*06 (P<.02), and -DQA1*03 (P<.003) was significantly different between the responsive and the nonresponsive groups. HLA-DQA1 allelic combination analysis showed that the frequencies of DQA1*03 and *05 had a high RR value in patients with sudden sensorineural hearing loss (RR = 4.1, P<.003) and in patients in the nonresponsive group (RR = 8.9, P<.001), compared with the controls. CONCLUSION: The presence of HLA class II alleles may be a useful genetic marker in forecasting a prognosis in Korean patients with sudden sensorineural hearing loss.

The clinical evaluations of pathophysiology for congenital middle ear cholesteatoma.

PURPOSE: Congenital middle ear cholesteatoma (CMEC) is less common than the acquired variety. Many theories have been put forward to explain the pathophysiology of CMEC; however, none of them have been convincingly proven thus far. This clinical study was performed to analyze the case reviews; assess the characteristic features of CMEC, and evaluate the correlation between those features and the pathophysiology of CMEC. METHODS: The medical records of patients who underwent otologic procedures at the hospitals of the Catholic University, Seoul, Korea, from January 1993 to November 1998 have been reviewed. RESULTS: Of the 14 patients, 3 had cystic lesions isolated to the anterosuperior quadrant of the mesotympanum. The lesions were easy to remove and did not affect the patients' hearing. Eleven patients had more extensive disease with posterior mesotympanum involvement; the lesions were large, were often too extensive to indicate a formative site, and caused ossicular damage. CONCLUSION: CMEC manifests in 2 distinctive forms according to the formation site. This distinctive formation site suggests that the pathophysiology of posterior lesions may be different from that of anterior ones. For early diagnosis of CMEC, a screening program should be established for children to prevent more extensive disease.

Distribution of HLA-A, -B and -DRB1 alleles in patients with sudden sensorineural hearing loss.

This study was performed to investigate the association between human leukocyte antigen (HLA) and susceptibility to sudden sensorineural hearing loss in the Korean population. HLA-A and HLA-B typing using a standard microlymphocytotoxicity technique and HLA-DRB1 genotyping were performed in 35 patients with sudden sensorineural hearing loss and in 206 healthy controls. Prednisone (usual dose 60 mg/day) was administered for 6 days and tapered for an additional 4-6 days. Both initial hearing levels at the onset of deafness and final hearing levels after treatment were examined and evaluated for association with HLA alleles. The frequency of HLA-DRB1*14 was increased in patients with sudden sensorineural hearing loss compared with controls (relative risk [RR] = 2.7, p = 0.016). The frequencies of HLA-A2, -A31, -B52, -B61, -DRB1*04, -DRB1*11 and -DRB1*12 were slightly higher than in the controls, but did not reach statistical significance. When an association between the treatment results and HLA alleles was also evaluated, the frequency of HLA-DRB1*04 was found to be increased in the patients who did not respond to steroid treatment compared with both patients who responded well to steroid (50%, vs 16%, p = 0.034) and controls (RR = 3.0, p = 0.046). These results suggest that there is an association between HLA-DRB1*14 and disease susceptibility and that the presence of HLA-DRB1*04 may be an useful marker for predicting a poor prognosis in Korean patients with sudden sensorineural hearing loss.

Effects of platelet-derived growth factor-AA on the healing process of tympanic membrane perforation.

PURPOSE: Platelet-derived growth factor basic 30-kD disulfide-bonded dimer of A and B chains (PDGF-AA, PDGF AB, PDGF-BB) and a cytokine, promoting wound healing by its mitogenicity for fibroblast and by stimulating the production of fibronectin and hyaluronic acid. This article investigates the effect of PDGF on the healing process of tympanic membrane (TM) perforation. MATERIALS AND METHODS: The pars tensa of the posterior aspect of the TM of rats was excised and treated with 2 microg of PDGF-AA or placebo. The animals were killed at 3, 5, 7, 9, 11, 15, and 28 days after operation. The healing process of TM perforation was observed with a telescope and light microscope. The temporal bones were also immunohistochemically examined for PDGF-alpha receptor (PDGF-R(alpha)) and fibronectin. RESULTS: All PDGF-AA-treated TM were completely closed by 5 days after surgery, whereas some of the placebo-treated TM were not closed at 15 postoperative days. PDGF-AA induced the most prominent proliferation of the connective tissue by 9 postoperative days, after which the growth of the connective tissue decreased. By the 4th postoperative week, the PDGF-treated TM were slightly thicker than normal TM. An intense expression of fibronectin was detected in the connective tissue layer of the TM that were treated with PDGF-AA. PDGF-R(alpha) was expressed in the epithelial layer of both the PDGF-treated and control TM. CONCLUSION: These results show that PDGF-AA speeds up the healing process of TM defect, improves the rate of healing, and prevents atrophic changes in the healed TM by promoting the connective tissue growth. The use of PDGF-AA can be an effective alternative to surgery for managing TM perforations.

Size assessment of adenoid and nasopharyngeal airway by acoustic rhinometry in children.

Adenoid hypertrophy is known as the most common cause of nasal obstruction in children; thus, adenoidectomy with, or without, tonsillectomy is one of the most commonly performed surgical procedures in the paediatric population. Although many methods have been suggested, few studies have reported on how to assess adenoid size, pre-operatively. Acoustic rhinometry is an objective technique as well as a non-invasive method, which can be easily used in young children. This study confirmed that acoustic rhinometry is a non-invasive and objective technique for assessing the geometry of the nasal cavity and nasopharynx. Forty children were evaluated using symptomology, two different radiological measurements and acoustic rhinometry; the results were compared with endoscopic findings. Clinical symptoms and A/N ratio measured with Fujioka's method significantly correlated with the endoscopic assessment findings (r = 0.769 and 0.604 respectively). Significant increases in the cross-sectional area and volume of the nasopharynx were observed at the adenoid notch after adenoidectomy (p < 0.005 and p < 0.005, respectively). Acoustic rhinometry showed a high degree of correlation of which adenoid occupied the nasopharyngeal airway under endoscopic examination (r = 0.771). Thus, the study concluded that acoustic rhinometry can be as good an objective method for measuring adenoid sizes as endoscopy and can be used as one of the pre-operative examination tools for adenoidectomy.

Recurrent Twiddler's syndrome in a nonthoracotomy ICD system despite a Dacron pouch.

Twiddler's syndrome is a highly recognized yet rare complication of pacemaker and cardioverter defibrillator (i.c.d.) implantation. We present a case in which persistent generator rotation resulted in lead dislodgment and inappropriate shocks in an initial ICD and recurrent lead fracture in a second ICD system. This case is unusual in that even with extensive surgical precautions including use of a Dacron pouch, generator rotation could not be prevented. Submuscular implantation and use of a smaller generator may prevent Twiddler's syndrome.