Clinical and technical factors in endoscopic skull base surgery associated with reconstructive success.

BACKGROUND: In this study, we identified key discrete clinical and technical factors that may correlate with primary reconstructive success in endoscopic skull base surgery (ESBS). METHODS: ESBS cases with intraoperative cerebrospinal fluid (CSF) leaks at four tertiary academic rhinology programs were retrospectively reviewed. Logistic regression identified factors associated with surgical outcomes by defect subsite (anterior cranial fossa [ACF], suprasellar [SS], purely sellar, posterior cranial fossa [PCF]). RESULTS: Of 706 patients (50.4% female), 61.9% had pituitary adenomas, 73.4% had sellar or SS defects, and 20.5% had high-flow intraoperative CSF leaks. The postoperative CSF leak rate was 7.8%. Larger defect size predicted ACF postoperative leaks; use of rigid reconstruction and older age protected against sellar postoperative leaks; and use of dural sealants compared to fibrin glue protected against PCF postoperative leaks. SS postoperative leaks occurred less frequently with the use of dural onlay. Body-mass index, intraoperative CSF leak flow rate, and the use of lumbar drain were not significantly associated with postoperative CSF leak. Meningitis was associated with larger tumors in ACF defects, nondissolvable nasal packing in SS defects, and high-flow intraoperative leaks in PCF defects. Sinus infections were more common in sellar defects with synthetic grafts and nondissolvable nasal packing. CONCLUSIONS: Depending on defect subsite, reconstructive success following ESBS may be influenced by factors, such as age, defect size, and the use of rigid reconstruction, dural onlay, and tissue sealants.

[Study on the salt intake of Jiaodong residents after the salt reduction intervention program and its correlation with blood pressure].

In 2016, the median of 24-hour urinary sodium, sodium-to-potassium ratio, and sodium intake per capita of residents in Jiaodong area of Shandong Province were 161.7 (IQR:120.5, 218.9) mmol/d, 3.4 (IQR: 2.4, 5.0) and 9.5 (IQR:7.1, 12.8) g/d, all were lower than that of 2011[193.3(IQR:149.2,243.3)mmol/d, 4.5(IQR:3.3,5.9), 11.3(IQR:8.7, 14.2)g/d] (P<0.05); the median of 24-hour urinary potassium was 47.2 (IQR:34.4, 66.5) mmol/d, higher than that of 2011[42.9(IQR:33.6,56.0)] (P<0.05); the proportion of salt intake per capita per day exceeding 5 g (89.9%) was lower than that in 2011 (90.0%). The results of the generalized linear regression model showed that the 24-hour urinary sodium was positively correlated with systolic and diastolic blood pressure [ß values were 0.04 (95%CI: 0.00, 0.07), 0.03 (95%CI 0.01, 0.05), respectively], and the sodium-to-potassium ratio was positively correlated with diastolic blood pressure [ß(95%CI): 0.78 (0.09, 1.47)].

[Risk factors and impacts on prognosis of ultrasound lung comets in patients undergoing hemodialysis].

Objective: To investigate the risk factors of ultrasound lung comets and its impact on the survivals of patients undergoing hemodialysis. Methods: One hundred and forty-two patients on hemodialysis (Male 76, female 66) were divided into three groups according to the score of lung comets (mild: ≤14 comets; moderate: 15 to 30 comets; severe: >30 comets). Seventy-two healthy subjects examined by lung ultrasound serve as a control. Pulmonary artery systolic pressure and left ventricular ejection fraction (LVEF) were assessed by Doppler ultrasonography. High-sensitivity C-reactive protein (hsCRP) and tumor necrosis factor (TNF)-α were measured by the automatic analyzer and enzyme-linked immunosorbent assay. Results: With the increasing age of the patients, lung comets scores increased (P<0.05). There were significant differences in TNF-α (P<0.05), interdialytic weight gain (IDWG) (P<0.05), pulmonary artery systolic pressure and LVEF (P<0.05) among three groups. In multivariate linear regression, the lung comets score was positively related to multiple clinical variables including New York Heart Association (NYHA) classification (P=0.023), hsCRP (P=0.042), TNF-α (P<0.001), IDWG (P=0.031), and pulmonary artery systolic pressure (P<0.001). In the multivariate Cox proportional hazards models, lung comets score was independent risk factor for death (P=0.001). In Kaplan-Meier survival analysis, the risk of all-cause mortality increased in parallel with lung comets score, and patients with lung comets score (>30 comets) were at highest risk of death among all three groups (log-rank test χ(2)=12.73, P=0.001). Conclusion: Lung comets is associated with inflammation, pulmonary artery systolic pressure/volume overload and heart function. Lung comets score represents the alterations of heart function and it may serve as a powerful predictor of all-cause mortality for hemodialysis patients.

The evolution of white matter microstructural changes after mild traumatic brain injury: A longitudinal DTI and NODDI study.

Neuroimaging biomarkers that can detect white matter (WM) pathology after mild traumatic brain injury (mTBI) and predict long-term outcome are needed to improve care and develop therapies. We used diffusion tensor imaging (DTI) and neurite orientation dispersion and density imaging (NODDI) to investigate WM microstructure cross-sectionally and longitudinally after mTBI and correlate these with neuropsychological performance. Cross-sectionally, early decreases of fractional anisotropy and increases of mean diffusivity corresponded to WM regions with elevated free water fraction on NODDI. This elevated free water was more extensive in the patient subgroup reporting more early postconcussive symptoms. The longer-term longitudinal WM changes consisted of declining neurite density on NODDI, suggesting axonal degeneration from diffuse axonal injury for which NODDI is more sensitive than DTI. Therefore, NODDI is a more sensitive and specific biomarker than DTI for WM microstructural changes due to mTBI that merits further study for mTBI diagnosis, prognosis, and treatment monitoring.

Role of short RNAs in gene silencing.

Recent research has revealed the existence of an elegant defence mechanism in plants and lower eukaryotes. The mechanism, known in plants as post-transcriptional gene silencing, works through sequence-specific degradation of RNA. It appears to be directed by double-stranded RNA, associated with the production of short 21-25 nt RNAs, and spread through the plant by a diffusible signal. The short RNAs are implicated as the guides for both a nuclease complex that degrades the mRNA and a methyltransferase complex that methylates the DNA of silenced genes. It has also been suggested that these short RNAs might be the mobile silencing signal, a suggestion that has been challenged recently.

Detection of chromosome 11q13 amplification in head and neck cancer using fluorescence in situ hybridization.

BACKGROUND: Head and neck squamous cell carcinoma (HNSCC) remains a cancer with one of the lowest five-year survival rates. Despite a better understanding of the disease and recent advances in diagnosis and treatment, survival rates for HNSCC patients have not improved. Chromosomal abnormalities are common in HNSCC, and aberrations of chromosome 11q13 have been correlated with a poor prognosis. MATERIALS AND METHODS: In this study we utilized fluorescence in situ hybridization (FISH) to determine the incidence of 11q13 amplification in twenty primary HNSCC tumors. INT-2 was used as the 11q13 probe, and 9 and 11 centromeric probes were used as controls. RESULTS: Polysomy, greater than two copies of chromosome 11, was found in 2 of 20 tumors. INT2 (11q13) amplification was found in 3 other tumors. CONCLUSIONS: These preliminary studies indicate tht analysis of a larger sample of tumors using FISH may yield important diagnostic and prognostic information about head and neck tumors.

Efficacy and morbidity of partial laryngectomy and postoperative radiation therapy.

In recent years, we have seen increasing use of partial laryngectomies for larger lesions that were previously treated by total laryngectomy. The resultant closer margins have made postoperative radiation therapy an important adjuvant treatment to conservation laryngeal surgery. We review the University of California, Los Angeles, experience with combination partial laryngectomy and postoperative radiation therapy between 1973 and 1987 for treatment of carcinoma of the larynx. Twenty-four such patients who underwent partial laryngectomies and postoperative radiation therapy are examined. Techniques of treatment, complications, and the functional ability of the remaining larynx are discussed. The locoregional control rate at 5 years was 80%. Risk factors associated with an increased risk of recurrence were positive margins, vascular invasion, and extranodal spread. There were no major problems with postoperative wound healing or airway management during the radiation treatment. Vocal and swallowing function were well preserved in most cases. We conclude that combination partial laryngectomy and radiation therapy permits preservation of laryngeal function without serious complications, and therefore is an effective treatment for selected patients with carcinoma of the larynx.

Combined radiotherapy with planned neck dissection for small head and neck cancers with advanced cervical metastases.

BACKGROUND: We have previously described our treatment algorithm for patients with small head and neck cancers with advanced cervical metastases (stage N2 or greater). Primary radiotherapy is given to the primary site and neck, followed 6 weeks later with endoscopy and biopsy of the primary site. If biopsy of the primary site is negative by frozen section, an immediate neck dissection is performed even when no clinical residual neck disease is present. Our initial review found that 36% of patients with a complete clinical response to radiotherapy had positive nodes on histological examination. STUDY DESIGN: Retrospective. METHODS: The medical records of 71 patients treated at UCLA Medical Center from 1986 to 1999 by this algorithm were reviewed. RESULTS: After radiotherapy, 69 of 71 patients had a complete response at their primary site. Forty-two patients had a complete clinical response in the neck. Seventy-one neck dissections were performed. Overall, 31 of 71 neck dissections (44%) had positive nodes. Among the 42 patients with a complete response to radiotherapy, 13 (31%) had positive histological nodes. Among the 29 patients with a partial response to radiotherapy, 17 (59%) had positive nodes. Follow-up and incidence of neck recurrence are discussed. CONCLUSION: Planned neck dissection for advanced cervical metastases remains controversial for patients with a complete clinical response to radiotherapy. However, our results suggest that clinical assessment after radiotherapy cannot assure the absence of neck disease. Until there are reliable methods to distinguish which patients are truly free of neck disease, we believe the benefits of a planned neck dissection outweigh the low morbidity of this procedure.

Antisense cyclin D1 enhances sensitivity of head and neck cancer cells to cisplatin.

OBJECTIVES: Cyclin D1 is a cell cycle regulatory factor that modulates a critical step in cell cycle control. Cyclin D1 is overexpressed in a significant proportion of head and neck cancers and correlates with a poor prognosis. Abrogation of cyclin D1 action through antisense cyclin D1 shows promise as an antitumor therapy, with an inhibitory effect in head and neck squamous cell carcinoma both in vitro and in vivo. The suppressive effect of antisense cyclin D1 in head and neck cancer xenografts in nude mice is incomplete, however, suggesting that combination with another antitumor agent is necessary for complete tumor eradication. Cisplatin is a widely used chemotherapeutic agent in head and neck cancer, and is particularly effective in combination with radiation therapy. In this study, we investigate whether antisense cyclin D1 enhances the sensitivity of head and neck cancer cells to cisplatin. Such an enhancement of sensitivity would suggest that combination therapy using antisense cyclin D1 and cisplatin would be an effective treatment modality for head and neck cancer. STUDY DESIGN: Antisense cyclin D1 was transfected into the head and neck squamous cell carcinoma cell line CCL23 using a plasmid vector. Both the parental CCL23 cells and the antisense cyclin D1-transfected CCL23 cells (CCL23AS) were treated with cisplatin at increasing concentrations. The dosage of cisplatin ranged from 1 microg/mL to 10 microg/mL. Initial exposure to cisplatin was for 2 hours, with increasing exposure times in succeeding experiments. Cell viability assays were done following cisplatin exposure. Dose response curves for the two cell lines were plotted and compared. Western blot analyses were done on the cisplatin-treated cell lines to determine levels of cyclin D1 expression. RESULTS: Increasing concentrations of cisplatin resulted in significantly higher rates of cell killing in the antisense cyclin D1-transfected cells than in the parental cells. The ID50 values for the parental CCL23 cells and the antisense cyclin D1-transfected CCL23 cells were 7 microg/mL and 3 microg/mL, respectively, indicating significant enhancement of sensitivity to cisplatin in the antisense cyclin D1-transfected cells. Western blot analyses demonstrated decreased expression of cyclin D1 in the CCL23AS cells with increasing doses of cisplatin, compared with the parental CCL23 cells. CONCLUSIONS: Antisense cyclin D1-transfected CCL23 cells demonstrate an enhanced sensitivity to the effects of cisplatin compared with the parental cell line. Although the mechanism for this phenomenon is not completely understood, the data suggests the potential use of combination therapy using antisense cyclin D1 and cisplatin for head and neck cancers. While neither agent alone can completely eradicate head and neck cancers, the synergistic effect of the two may be an effective therapeutic protocol for refractory head and neck cancers. Future investigation into the combination of antisense cyclin D1 with cisplatin for treatment of head and neck cancer is needed.

Early experience with percutaneous tracheotomy.

Early reports of a percutaneous dilatational technique for tracheotomy tube placement have been encouraging. This method uses a needle for placement into the trachea, a J-tipped guidewire, and progressively larger dilators to widen the stoma for insertion of a tracheotomy tube. A prospective study was undertaken to assess the efficacy of this percutaneous tracheotomy technique. Serious complications were encountered, including difficulty with dilatation, excessive bleeding, false passage of the tracheotomy tube, and death. Because of this unacceptable morbidity, the study was terminated after enrollment of only seven patients. Percutaneous tracheotomy is a dangerous procedure with potential for catastrophic complications. These complications were encountered by third- and fourth-year surgical residents performing the procedure under close supervision. If the puncture technique is further popularized and attempted by untrained physicians, these authors predict many disasters.

Increased expression of transforming growth factor-beta1, acidic fibroblast growth factor, and basic fibroblast growth factor in fetal versus adult fibroblast cell lines.

OBJECTIVES: Fetal wound healing occurs without scar tissue formation. Differences in growth factor expression between fetal and adult fibroblasts have been explored. Recently we used RNA expression studies to demonstrate that transforming growth factor (TGF)-beta1, acidic fibroblast growth factor (alpha-FGF), and basic fibroblast growth factor (beta-FGF) could be detected in both fetal and adult fibroblast cell lines. In addition, adult fibroblasts showed greater relative expression of these growth factors than fetal fibroblasts. The aim of this study was to identify the level of protein expression in fetal and adult fibroblasts. STUDY DESIGN/METHODS: Fetal and adult fibroblasts were grown in culture using standard and serum-free media. After protein extraction, Western blot studies were performed to detect the presence and amount of TGFbeta-1, alpha-FGF, and beta-FGF growth factor proteins. beta-Tubulin was used as a control. RESULTS: TGFbeta-1, alpha-FGF, and beta-FGF proteins were detected in fetal and adult fibroblasts grown in standard and serum-free media. The fetal fibroblasts showed higher levels of all three growth factor proteins compared with the adult fibroblasts. CONCLUSIONS: Western blot studies suggest higher levels of TGFbeta-1, alpha-FGF, and beta-FGF expression in fetal fibroblasts. It is clear that significant differences exist in the expression/production of these growth factors and it seems likely that further study of these differences will help elucidate the unique healing capabilities of fetal fibroblasts.

Characterization of lymphocyte subpopulations in Warthin's tumor.

Cell suspensions from six Warthin's tumors (WTs) were characterized with fluorescence-labeled cell cytometry. WT lymphocyte subsets were identified with monoclonal antibodies directed against lymphocyte-associated cell antigens including T lymphocyte subsets, B lymphocytes, and natural killer (NK) cells. Results showed that T cell proportions were 58% and B cell proportions were 39%. The T cell helper:cytotoxic-suppressor ratio was 5.7:1 and the B to T cell ratio was 0.8:1. NK cells represented 1.3% of cells. When compared to peripheral blood lymphocytes (PBLs) in the same patients, statistically significant differences were noted between PBLs and WT lymphocytes in the percentage of B lymphocytes (P < .01), T cytotoxic-suppressor lymphocytes (P < .02), NK cells (P < .01), and in the ratios of B to T lymphocytes (P < .01) and T helper to T cytotoxic-suppressor lymphocytes (P < .03). Comparing these data to retrospective data on lymphocyte distribution in normal and reactive lymph nodes, the epithelial component does not appear to exert a local effect on the lymphoid component of WT.

Extranodal Rosai-Dorfman disease of the head and neck.

Rosai-Dorfman disease is a rare, idiopathic, benign histiocytic proliferation usually seen in younger patients. Massive lymphadenopathy most commonly involves the cervical lymph nodes, with a predominant infiltration of sinusoidal histiocytes. Nearly half of the patients will have extranodal involvement, 75% occurring in sites in the head and neck. Three cases of extranodal Rosai-Dorfman disease of the head and neck involving the nose, paranasal sinuses, and parotid gland are presented. The clinical presentation, histologic characteristics, radiographic findings, and treatment of the disease are discussed. Because of the scarcity of cases, the clinical and histopathologic features of this disease may be overlooked. Familiarity with its relatively frequent clinical manifestations in the head and neck, as well as with the diagnostic histopathology, should preclude confusion with other disease entities.

Clinical application of fluorescence in situ hybridization for chromosome 11q13 analysis in head and neck cancer.

OBJECTIVE: Squamous cell carcinoma of the head and neck (HNSCC) still has one of the lowest 5-year survival rates. Despite advances in diagnosis, treatment, and research, survival rates have not improved in recent years. This report examines the utility of fluorescence in situ hybridization (FISH) in detecting chromosome 11q13 amplification in HNSCC and in evaluating the correlation between 11q13 amplification and tumor behavior. STUDY DESIGN: This study used FISH to determine the incidence of 11q13 amplification in 20 HNSCCs and 10 normal controls from the same patients. Tumor touch preparations and paraffin-embedded tissues from the same patient samples were used for comparative analysis. Both single and dual color FISH was performed. METHODS: Repetitive chromosome 11 specific alpha satellite DNA probe and chromosome 11q13 specific probe cyclin D1 were used for the FISH analysis. RESULTS: Experiments revealed amplification of chromosome 11q13 in three fresh touch preparations. FISH on paraffin tissues showed amplification in two additional samples. Intensity of amplification, as high as 20 copies per nucleus, was observed in paraffin preparations, whereas a maximum of only six copies was seen in fresh preparations. Amplification was not detected in any of the normal samples. All five cases with 11q13 amplification had metastases and four of these were from poorly differentiated tumors. In the nonamplified cases, 5 of 15 had metastases and 2 of 15 was poorly differentiated. CONCLUSIONS: The present study indicates that FISH is a useful technique for detecting molecular changes such as amplification of chromosome 11q13 in HNSCC. FISH in paraffin preparations allows for accurate measurement of intensity of amplification and makes it possible for the evaluation of a large collection of archival material. The data also suggest that 11q13 amplification is correlated with poorly differentiated tumors and metastasis. Thus FISH has the potential to be a valuable diagnostic/prognostic tool in head and neck cancers.

Open bedside tracheotomy in the intensive care unit.

OBJECTIVE: To demonstrate that open bedside tracheotomy is an efficient, safe, and cost-effective procedure. STUDY DESIGN: Retrospective review of more than 200 open bedside tracheotomies performed at UCLA Medical Center, Harbor-UCLA Medical Center, and West Los Angeles VA Medical Center from 1995 to 1998. METHODS: The only personnel required for the procedure were an attending or senior resident and a junior resident or intern, as well as the respiratory therapist to withdraw the endotracheal tube. No anesthetist or scrub nurse was present for any of the procedures. The procedure took an average of 15 to 25 minutes. Patients were followed for 30 days after surgery to determine the incidence of complications. RESULTS: The incidence of major complications related to the procedure, including hemorrhage and myocardial infarction, was less than 1%. The incidence of minor complications, including moderate bleeding at the tracheotomy site, was 4%. Overall mortality within 30 days was 8%, but was not related to the tracheotomy for any patients in this series. The charge for the procedure was $233 for the tracheotomy tube supplies and instruments. This cost compares favorably with an average charge of more than $3000 for the procedure in the operating room and about $1000 for a percutaneous tracheotomy kit. CONCLUSION: Review of our experience demonstrates that open bedside tracheotomies can be performed more efficiently and economically than operating room tracheotomies. The safety of this procedure is comparable to percutaneous tracheotomy but at a decreased cost.

Nitric oxide improves cisplatin cytotoxicity in head and neck squamous cell carcinoma.

OBJECTIVE: To test whether nitric oxide (NO) enhances the cytotoxicity of cisplatin in a head and neck squamous cell carcinoma (HNSCC) cell line. BACKGROUND: Cisplatin is one of the most frequently used chemotherapeutic agents in the treatment of HNSCC. NO has been shown to play an important role in regulating tumor growth. Previous studies demonstrate that NO can enhance the cytotoxicity of cisplatin in Chinese hamster lung fibroblasts. In this report, we examined the in vitro interaction of NO and cisplatin in a HNSCC cell line. MATERIALS AND METHODS: CCL23 cells were pretreated with three different NO donors: PAPA/NO (t 1/2 = 15 min), DPTA/NO (t 1/2 = 3 h), and DETA/NO (t 1/2 = 20 h). The cells were rinsed and exposed for 6 hours to a culture medium containing cisplatin. Cell survival and LD50 of cisplatin were calculated with and without NO pretreatment. RESULTS: PAPA/NO and DPTA/NO did not show any cytotoxic activity and did not change the LD50 of cisplatin. DETA/NO when used alone resulted in 25.6% cell death at its peak dose (100 microM). Pretreatment with DETA/NO resulted in almost a threefold reduction of the LD50 of cisplatin (6.8 vs. 2.4 microg/mL). Pretreatment with DETA/NO sensitized the HNSCC cells to subsequent cisplatin activity (two-sided P =.00016). CONCLUSION: Pretreatment of HNSCC cells with long-acting NO donors enhances cisplatin activity. Short- and medium-acting NO donors do not exert a toxic effect and do not augment the activity of cisplatin. NO agonists should be considered in the future as a possible adjunct to cisplatin in the treatment of HNSCC. Further studies with animal models are necessary to further clarify this relationship.

Extranodal American Burkitt's lymphoma of the head and neck.

Burkitt's lymphoma was first described in 1958 as a mandibular malignancy found in African children. The American, or nonendemic, form of the disease differs from the African form in that the tumor most often presents with abdominal or bone marrow involvement. Head and neck manifestations of American Burkitt's lymphoma are encountered in less than a quarter of the reported cases and usually present as cervical adenopathy. A review of all cases of American Burkitt's lymphoma treated at the UCLA Medical Center, Los Angeles, Calif, from 1971 to 1989 revealed an unusual subset of seven patients with American Burkitt's lymphoma of the head and neck who presented with extranodal disease of the soft tissues or bones of the face. The medical literature was reviewed to compile similar cases of extranodal American Burkitt's lymphoma. The clinical and pathologic features of these cases, their radiographic findings, and treatment are described.

Deletion analysis of the p16/CDKN2 gene in head and neck squamous cell carcinoma using quantitative polymerase chain reaction method.

BACKGROUND: Recently, the p16/CDKN2/MTS1 gene in the 9p21-22 region has been offered as a candidate tumor suppressor gene. We examined the frequency of hemizygous and homozygous deletions of p16/CDKN2 in head and neck squamous cell carcinoma (HNSCC) using a quantitative polymerase chain reaction (PCR) method. DESIGN: Twenty-one HNSCC and 12 corresponding normal DNA samples were examined for deletion of p16/ CDKN2 using PCR amplification and fluorescent quantification of DNA. All tumor and normal DNA samples were also amplified with fluorescein-labeled primers for a control DNA marker on chromosome 8p (D8S265). The ratios of the observed fluorescence of the p16/CDKN2 and 8p PCR products were compared. SETTING AND PARTICIPANTS: Patients with HNSCC scheduled to undergo surgical resection of their tumors were recruited. After the specimen was removed, a portion of the tissue was snap frozen for further DNA extraction. RESULTS: Eight tumors (38%) had p16/CDKN2-D8S265 ratios of greater than 0.75; 8 tumors (38%), from 0.25 to 0.75; and 5 tumors (24%), of less than 0.25, the average ratio in this last group being 0.06. CONCLUSIONS: These ratios suggest a higher rate of homozygous deletion than previously reported and significant probable hemizygous deletion of the p16/CDKN2 gene in HNSCC.

Amyloidosis of the head and neck. A clinicopathologic study of the UCLA experience, 1955-1991.

OBJECTIVE: To characterize the otolaryngologic manifestations of amyloidosis; classify patients with amyloidosis by specific location and type of underlying disease; and compare disease presentation and long-term outcome in these patients. DESIGN: Retrospective review of biopsy specimens recorded as amyloidosis. SETTING: Tertiary care referral center serving a diverse patient base. PATIENTS: Of 141 patients with biopsy-verified amyloidosis who met the inclusion criterion for the study, 27 (19%) had head and neck manifestations. OUTCOME MEASURES: Good functional outcome and survival of patients with amyloidosis. RESULTS: The tongue was the most commonly affected site of the head and neck. Distinct differences exist in functional outcome and long-term survival in patients with the localized form of amyloidosis when compared with patients with systemic amyloidosis. CONCLUSION: Because amyloidosis often affects the head and neck, otolaryngologists need to be familiar with this disease.