Portable audiometric screening platforms used in low-resource settings: a review.

BACKGROUND: Millions of people across the world suffer from disabling hearing loss. Appropriate interventions lead to improved speech and language skills, educational advancement, and improved social integration. A major limitation to improving care is identifying those with disabling hearing loss in low-resource countries. OBJECTIVES: This review article summarises information on currently available hearing screening platforms and technology available from published reports and the authors' personal experiences of hearing loss identification in low-resource areas of the world. The paper reviews the scope and capabilities of portable hearing screening platforms, including the pros and cons of each technology and how they have been utilised in low-resource environments. CONCLUSION: Portable hearing screening tools are readily available to assess hearing loss in low-resource areas. Each technology has advantages and limitations that should be considered when identifying the optimal methods to assess needs in each country.

Educational workshops with graduates of the University of Cape Town Karl Storz Head and Neck Surgery Fellowship Program: a model for collaboration in outreach to developing countries.

The University of Cape Town Karl Storz Head and Neck Surgery Fellowship is the only head and neck surgery fellowship in Sub-Saharan Africa. This article briefly describes this fellowship and outlines the experience and ongoing collaborative efforts of members of the American Academy of otolaryngology-head and neck surgery with graduates of this program who are now building head and neck surgery programs in East Africa. This educational collaboration avoids many common pitfalls associated with short-term humanitarian outreach and represents a successful model for international collaborative educational efforts with head and neck surgeons in developing countries in Africa.

Nuclear factor-kappa B pathway and response in a phase II trial of bortezomib and docetaxel in patients with recurrent and/or metastatic head and neck squamous cell carcinoma.

BACKGROUND: Our previous study has shown that nuclear factor-kappa B (NF-kappaB)-signaling pathway was associated with a higher rate of recurrence in head and neck squamous cell carcinoma (HNSCC). The combination of bortezomib, an NF-kappaB inhibitor by inhibition of proteasomes, plus docetaxel was assessed for efficacy and toxicity. MATERIALS AND METHODS: Patients with recurrent and/or metastatic HNSCC were enrolled on a phase II bortezomib/docetaxel trial (bortezomib 1.6 mg/m(2) and docetaxel 40 mg/m(2) on days 1 and 8 of a 21-day cycle). Response was assessed using RECIST. Tissue specimens were evaluated for the presence of human papillomavirus (HPV) and expression of NF-kappaB-associated genes. RESULTS: Twenty-one of 25 enrolled patients were assessable for response; one partial response (PR, 5%), 10 stable disease (SD, 48%) and 10 progressive disease (PD, 48%). Patients with PR/SD had significantly longer survival compared with patients with PD and the regimen was well tolerated. Only one of 20 tumors was positive for HPV. Patients with PD had higher expression of NF-kappaB and epidermal growth factor receptor-associated genes in their tumors by gene expression analysis. CONCLUSION: Further understanding of treatment resistance and interactions between bortezomib and docetaxel may provide novel approaches in managing HNSCC.

Microsatellite mutations in buccal cells are associated with aging and head and neck carcinoma.

Carcinogen exposure from tobacco smoking is the major cause of upper aerodigestive tract cancer, yet heavy smokers only have about a 10% life-time risk of developing one of these cancers. Current technologies allow only limited prediction of cancer risk and there are no approved screening methods applicable to the general population. We developed a method to assess somatic mutational load using small-pool PCR (SP-PCR) and analysed mutations in DNA isolated from cells obtained by mouth rinse. Mutation levels in the hypermutable tetranucleotide marker D7S1482 were analysed in specimens from 25 head and neck squamous carcinoma (HNSCC) cases and 31 controls and tested for associations with age, smoking history and cancer status. We found a significant association between mutation frequency and age (P=0.021, Generalized Linear Model (GLM), N=56), but no influence of smoking history. Cases had higher mutation frequencies than controls when corrected for the effects of age, a difference that was statistically significant in the subgroup of 10 HNSCC patients who were treated with surgery only (P=0.017, GLM, N=41). We also present evidence that cancer status is linked to levels of nonunique, and presumably clonally derived, mutations in D7S1482. Insertion mutations were observed in 833 (79%) of 1058 alleles, of which 457 (43%) could be explained by insertion of a single repeat unit; deletion mutations were found in 225 (21%) of tested alleles. In conclusion, we demonstrate that the sensitive detection of single molecule mutations in clinical specimens is feasible by SP-PCR. Our study confirms an earlier report that microsatellite mutations increase with age and is the first to provide evidence that these mutations may be associated with cancer status in individual subjects.

Voice gains following thyroplasty may improve over time.

Type I thyroplasty for unilateral vocal fold paralysis restores voice. The purpose of this study was to evaluate measures of voice before thyroplasty, and at 3 months and 1 year after surgery. Of interest was whether vocal improvement in the first weeks after surgery was maintained or even enhanced over time. A total of 40 patients with unilateral paralysis underwent type I thyroplasty with or without arytenoid adduction. Perceptual, acoustic and aerodynamic measures of voice were studied. Perceptual analysis determined that optimal postoperative voice quality evolved over the first year. Acoustic indices of perturbation demonstrated progressive improvement over 12 months, whereas pitch and intensity ranges were increasingly extended. Postoperative glottal flow rates were normalized and phonation times were significantly longer, with benefits maintained over time. All perceptual, aerodynamic and acoustic measures of voice were improved 3 months after thyroplasty, with many measures further improved at 1 year. Such findings provide evidence that voice outcome progressively evolves over the first 12 months after surgery.

Voice outcome following thyroplasty in patients with cancer-related vocal fold paralysis.

Medialization laryngoplasty restores voice in patients with unilateral laryngeal paralysis. Of question was whether patients with vocal fold paralysis resulting from cancer or its treatment had as good a post-operative voice result as patients with vocal fold paralysis of benign etiology. The purpose of the present study was to compare post-operative perceptual, acoustic, aerodynamic, and quality of life data in these two patient groups. Twenty-eight patients with vocal fold paralysis secondary to malignancy or its treatment were age and gender-matched with patients with paralysis resulting from benign origin. Pre- and post-operative perceptual judgments of pitch, loudness and quality were rated independently by two speech-language pathologists. A digital audiotape of the patient's voice was analyzed using Soundscope software. Fundamental frequency, conversational intensity and perturbation were evaluated. Glottal flow rates in propositional speech, phonation times and extent of pitch and loudness ranges were also measured. Three quality of life surveys, the Short Form-36 general health survey, the Voice Handicap Index, and the Voice Outcomes Study were administered. Results of voice testing indicated that perceptual, acoustic and aerodynamic data were significantly improved 3 months after thyroplasty in all patients regardless of whether they had a history of cancer. Quality of life data, however, distinguished the two groups. In particular, the general health measure found a significant difference in physical functioning and overall vitality, although satisfaction with improved voice was equally appreciated in both patient groups. Of clinical significance is that though general health may differ, patients with cancer-related laryngeal paralysis can expect to have as good a voice outcome following thyroplasty as patients with paralysis of benign etiology.

Quality-of-life assessment in patients with unilateral vocal cord paralysis.

OBJECTIVE: Our objective was to identify the impact of unilateral vocal cord paralysis (UVCP) on an individual's quality of life both before and after thyroplasty. STUDY DESIGN AND SETTING: This was a prospective observational outcome study of consecutive patients presenting to a laryngology clinic with UVCP. Participants received The Medical Outcomes Study Short Form 36-Item Health Survey (SF-36), the Voice Handicap Index (VHI), and the Voice Outcome Survey (VOS). Patients underwent medialization laryngoplasty with silastic, with or without arytenoid adduction. Outcome measures were repeated after surgery. RESULTS: A review of 45 patients at presentation revealed statistically significant reductions in quality of life as measured by each survey. Marked improvements were noted after surgery. CONCLUSIONS: Our preoperative data support a profile of significant general health-related and voice-related limitations caused by UVCP. Patient perceptions improved significantly after the surgical treatment of glottal insufficiency. SIGNIFICANCE: The SF-36, VHI, and VOS provide an important complement to traditional endpoints in the analysis of patients with UVCP.

Vagal paragangliomas.

The management of vagal paragangliomas is extremely challenging. Treatment of these lesions must be tailored individually for each patient. The best treatment modality depends on the patient's age and health and the size and extent of the tumor. This article discusses clinical presentation, multicentric and malignant vagal paragangliomas, evaluation, embolization, surgical management, and special considerations in the management of vagal paragangliomas.

Paraganglioma surgery: complications and treatment.

Paragangliomas are vasculature in nature and are surrounded by vital neurovascular structures. The extirpation of these lesions requires careful preoperative evaluation, meticulous surgical technique, and the aid of experienced skull base surgical and rehabilitative teams. When surgery is performed in this way, complications can be minimized, and the function of the upper aerodigestive tract can be protected.

Topoisomerase I inhibitors in the treatment of head and neck cancer.

Traditionally, the role of chemotherapy in the treatment of squamous carcinoma of the head and neck has been confined to patients with recurrent or metastatic disease who are deemed incurable with surgery or radiation therapy. Over the past decade, however, the role of chemotherapy has changed dramatically. The use of primary combined chemoradiation to preserve function or to enhance survival in patients with unresectable disease has become a standard approach. As the use of chemotherapy in squamous carcinoma of the head and neck has expanded, investigators have been interested in identifying new active agents. Topoisomerase I inhibitors, a new class of drugs, have been found to be active in a number of solid and hematologic malignancies. Three topoisomerase I inhibitors have been investigated in the treatment of metastatic or recurrent squamous carcinoma of the head and neck: 9-aminocamptothecin (9-AC), topotecan (Hycamtin), and irinotecan (CPT-11, Camptosar). Neither 9-AC nor topotecan has demonstrated clinically significant activity in the treatment of metastatic or recurrent squamous carcinoma of the head and neck. In contrast, irinotecan has demonstrated a modest overall response rate of 21.2% (95% confidence interval [CI] = 9%-38.9%), with a median survival of 214 days and a 1-year survival rate of 30.2%. The response and toxicity appear to be dose dependent. Further investigation of irinotecan in combination with other active agents and radiotherapy is warranted.

Voice outcome following lateral laryngotomy to remove teflon granuloma.

Formation of a Teflon granuloma may lead to progressive dysphonia and airway compromise. Excision of the granuloma by lateral laryngotomy allows preservation of the uninvolved lamina propria. A sternothyroid muscle flap or Silastic implant to medialize the vocal fold restores a straight glottal edge and optimizes voice production. Fifteen patients underwent removal of a Teflon granuloma via a lateral approach. Analysis of data revealed improved acoustic and aerodynamic parameters of voice following surgery. Indices of acoustic perturbation were reduced, and vocal pitch normalized. While the dynamic pitch range was unchanged, the capacity to vary loudness was enhanced. Flow rates in speech, abnormally elevated before surgery, normalized after the procedure, and phonation times were significantly longer. Perceptual and stroboscopic data confirmed that voices were improved, but not normal.

Lateral skull base surgery for glomus tumors: long-term control.

OBJECTIVE: The age of modern microsurgery has made resection of glomus tumors with extensive skull base involvement possible. Resection of extensive lesions is not without risk of major complication or new cranial nerve deficit. Because glomus tumors are rare and slow growing, data reflecting recurrence risk after resection using modern skull base techniques are lacking. These factors complicate the accurate definition of efficacy of surgical resection and its functional cost. The object of this review is to determine the current incidence of major complications, the surgical cranial nerve deficit, the long-term control rate, and the recurrence risk in patients undergoing surgical resection of glomus jugulare tumors. STUDY DESIGN: Retrospective case review. SETTING: Private practice tertiary referral center. PATIENTS AND INTERVENTIONS: One hundred seventy-six patients with glomus tumors underwent 182 lateral skull base resections between 1972 and 1998. MAIN OUTCOME MEASURES: Major complications, tumor recurrence, postoperative functional capacity, and factors affecting the incidence of each were assessed. RESULTS: Complete surgical control was achieved in 85% of cases. There were nine cases of recurrence, for a recurrent rate of 5.5% (9/164). Cerebrospinal fluid leakage occurred in 4.5% of cases with intracranial extension. New deficits for cranial nerves IX, X, XI, and XII occurred in 39%, 25%, 26%, and 21% of cases, respectively. Satisfactory functional recovery was achieved in an overwhelming majority of cases. The mortality rate was 2.7% (5/182). CONCLUSIONS: Surgical resection of glomus tumors is established as an effective technique with good functional outcomes and long-term control.

Outcomes assessment following treatment of spasmodic dysphonia with botulinum toxin.

Spasmodic dysphonia (SD), a disabling focal dystonia involving the laryngeal musculature, is most commonly treated by the intramuscular injection of botulinum toxin (BTX). Although the treatment is well tolerated and generally produces clinical voice improvement, it has never been statistically shown to alter the patient's perception of voice quality or general health. Declining resources for medical care mandate that treatment outcomes be documented. A prospective analysis of the effects of BTX on the patient's perception of voice and general health was undertaken. The Voice Handicap Index (VHI) and Short Form 36 (SF-36) surveys were administered to patients before treatment and 1 month after. Pretreatment and posttreatment scores were analyzed with a Student's t-test. On the VHI, improvements in the patients' perception of their functional, physical, and emotional voice handicap reached statistical significance (p < or = .0005). On the SF-36, patients had statistically significant improvements in mental health (p < or = .03) and social functioning (p < or = .04). Treatment of SD with BTX significantly lessened the patients' perception of dysphonia. In addition, it improved their social functioning and their perception of their mental health. These outcome measures justify the continued treatment of SD with BTX.

Increasing use of the scapula osteocutaneous free flap.

OBJECTIVES: To determine the appropriate use of the scapula osteocutaneous free flap (SOFF) and to document donor site morbidity. STUDY DESIGN: Retrospective review and prospective physical therapy evaluation. METHODS: A computer database of all free flap procedures performed at a single institution was created. Specific clinical and operative details from cases involving a bone flap were extracted from the database. Rates of usage of the various osteocutaneous flaps were compared over four successive 2-year intervals (1992-1999). A single physical therapist performed a structured evaluation of the donor site. RESULTS: Overall, 64 bone flap procedures were performed, of which 24 (37.5%) were SOFF procedures. The SOFF utilization has increased from 6.6% to 63.6%, while fibula and iliac crest utilization has fallen significantly. This is in part because of the greater versatility of the SOFF, with the possibility of separate skin paddles and adequate bone length. The mean cutaneous area harvested with the SOFF was 110 cm2 (range, 48-200 cm2) compared with 55.4 cm2 (range, 25-102 cm2) and 77.6 cm2 (range, 50-120 cm2) for the fibula and iliac crest, respectively. Mean bone flap lengths were 8.37, 7.65, and 10.1 cm, respectively, for the SOFF, fibula, and iliac crest. Dual skin paddles were used in 50% of the SOFF procedures versus 2.8% for the fibula flap procedures. There were no significant complications of the donor site in any patient, and there was only one flap failure (4.1%). Related to the SOFF, donor site morbidity was subjectively judged as "mild," for pain, mobility, and strength. There were no complaints of poor appearance of the donor site. Activities of daily living were judged as "not limited" or 'limited a little" in the majority of patients. Objective measurements of range of motion revealed an average reduction of 1 degree to 12 degrees in five different shoulder functions. Elbow and arm ranges of motion were not limited. Strength was minimally reduced in the shoulder, while the arm and forearm showed no reduction in strength. CONCLUSIONS: The SOFF is a versatile osteocutaneous free flap that can be used for a multitude of reconstructive problems. This and its relative lack of significant donor site morbidity have caused its use to increase significantly.

Sarcoidosis of the pediatric larynx.

Sarcoidosis is a chronic granulomatous disease of unknown pathogenesis. Reports of sarcoidosis are much less common in the pediatric literature than in the adult literature. The disease is usually systemic; rarely, however, single organs are affected. Isolated laryngeal involvement is an unusual presentation. We report a well-documented case of isolated laryngeal sarcoidosis in a 14-year-old white boy who presented to our institution with a 6-month history of dysphonia, dyspnea on exertion, and extremely sonorous snoring at night owing to his supraglottic airway disease. To our knowledge, this is only the second case of isolated laryngeal sarcoidosis reported in the pediatric literature. We review the literature and discuss the differential diagnosis, diagnostic evaluation, and treatment with carbon dioxide laser epiglottectomy and intralesional glucocorticoid deposition, which resulted in marked resolution of our patient's symptoms.

Long-standing lateral neck mass as the initial manifestation of well-differentiated thyroid carcinoma.

OBJECTIVE: To analyze the presentation, evaluation, and treatment of a subset of patients with well-differentiated thyroid carcinoma who present with a lateral neck mass and no palpable disease in the thyroid gland. STUDY DESIGN: A retrospective review of all patients undergoing thyroidectomy for malignancy. METHODS: A database of all thyroidectomies performed for malignancy by the Vanderbilt University Department of Otolaryngology-Head and Neck Surgery from 1992 to 1997 was created. Patients who presented with an isolated neck mass without evidence of palpable disease in the thyroid were selected for the study population. RESULTS: There were 60 cases of thyroid malignancy, with 14 cases (23.3%) that presented as isolated lateral neck mass. The characteristics of this group (compared with the population of all thyroid malignancies) include younger age at presentation (37.7 +/- 15.2 y vs. 49.8 +/- 15.6 y; Student t test: P = .019) and long-standing presence of symptoms (27.4 +/- 39.6 mo vs. 3.6 +/- 3.9 mo; P = .023). These patients generally presented from a referring facility after having an excisional biopsy, which was 100% accurate. Fine-needle aspiration is becoming more useful and was 66.7% accurate. Histological examination revealed cancer in the thyroid gland in all patients, 11 cases of papillary carcinoma, 2 follicular carcinomas, and one medullary carcinoma The mean size of the primary focus was 10.9 +/- 8.7 mm, with 29% demonstrating bilateral disease and 14% demonstrating multifocal disease in the ipsilateral gland. The neck specimens revealed an average of 5.3 +/- 3.2 metastatic nodes in levels II-IV and 3.9 +/- 4.6 metastatic nodes in the paratracheal region. CONCLUSION: Based on this patient population, the long-standing lateral neck mass in the young patient should raise the physician's index of suspicion for thyroid carcinoma Fine-needle aspiration should be used in conjunction with judicious excisional biopsy. The bilateral and multifocal nature of otherwise occult primary disease argues for total thyroidectomy in this setting.

Histologic findings of silastic medialization in the canine model.

OBJECTIVES: Examine the long-term histological effects of Silastic medialization using a canine model. STUDY DESIGN: Prospective study evaluating the effects of Silastic medialization in six canine larynges. METHODS: Two subject groups were evaluated. The first group of four dogs underwent recurrent laryngeal section followed by Silastic medialization on the paralyzed side and placement of a smaller implant on the mobile side. Two of the dogs were sacrificed at 9 months and two at 2 years. The second subject group consisted of two additional dogs who underwent Silastic medialization followed by removal of the implants at 7 months and were sacrificed 18 months later. The larynges were then processed, sectioned in the axial plane, and examined microscopically. RESULTS: Histological findings of both the mobile and paralyzed sides showed a thin (< 0.5 mm) fibrous capsule surrounding the implant and minimal inflammatory response. Vocal fold mobility was maintained in all cases in which the recurrent laryngeal nerve was left intact. Capsule formation and inflammatory response were similar in all cases. CONCLUSIONS: Minimal tissue reactivity over a prolonged period of time suggests that long-term results of Silastic medialization remain stable in paralyzed and mobile vocal folds for up to 2 years. Similar tissue response in the subjects in which the Silastic block was removed also suggests that Silastic medialization is a reversible procedure.

Surgical management of thyroid and parathyroid disorders.

This article begins with a discussion of thyroid anatomy and physiology and then proceeds with an in-depth study of the area of focus, thyroid and parathyroid disorders, including thyroid nodules and cancer, multinodular goiter, and hyperparathyroidism. Complications of thyroid and parathyroid surgery are discussed.