Objectives/Hypothesis: Audiometric patterns have been shown to indirectly provide information regarding the pathophysiology of presbycusis and be useful in the phenotyping of hereditary deafness. Study Design and Methods: Hospital-based cohort study of adults with presbycusis, comparing the association of audiometric patterns and polymorphisms of antioxidant enzymes that have been linked to presbycusis: GSTT1, GSTM1 and NAT2. All subjects underwent a clinical evaluation and completed questionnaires regarding ototoxicity and noise exposure. Pure-tone threshold audiometry was obtained and subjects' audiograms were classified into specific patterns. DNA was extracted from blood and the polymorphisms of GSTT1, GSTM1, and the NAT2 variants (NAT2* 5A; NAT2* 6A,B) were analyzed by PCR. Results: The audiometric patterns that were more prevalent in our cohort were High-Frequency Steeply Sloping or HFSS (33%), High-Frequency Gently Sloping or HFGS (31%), and Flat (27%), with other patterns being rare. We did not find a statistical significant effect of gender, age, hearing level, and ear side on the audiometric pattern. Subjects with mutant alleles for GSTT1 were more likely to have a HFSS audiogram than subjects with the wild type genotype. Conclusions: In this cohort, there was a similar prevalence for the three audiometric configurations HFSS, HFGS, and Flat, with other configurations being rare. Subjects with mutant alleles for GSTT1 were more likely to have a HFSS audiogram than subjects with the wild type genotype, suggesting that the basal turn of the cochlea is susceptible to GSTT1 regulated oxidative stress. However, further studies of audioprofiles with larger sample sizes may be needed to establish phenotype-genotype correlations in presbycusis. Laryngoscope, 2012

OBJECTIVEA proposed mechanism for presbycusis is a significant increase in oxidative stress in the cochlea. The enzymes glutathione S‐transferase (GST) and N‐acetyltransferase (NAT) are two classes of antioxidant enzymes active in the cochlea. In this work, we sought to investigate the association of different polymorphisms of GSTM1, GSTT1, and NAT2 and presbycusis and analyze whether ethnicity has an effect in the genotype‐phenotype associations.STUDY DESIGNCase‐control study of 134 DNA samples.SETTINGUniversity‐based tertiary care center.SUBJECTS AND METHODSClinical, audiometric, and DNA testing of 55 adults with presbycusis and 79 control patients with normal hearing.RESULTSThe GSTM1 null genotype was present in 77 percent of white Hispanics and 51 percent of white non‐Hispanics (Fisher's exact test, 2‐tail, P = 0.0262). The GSTT1 null genotype was present in 34 percent of control patients and in 60 percent of white presbycusis subjects (P = 0.0067, odds ratio [OR] = 2.843, 95% confidence interval [95% CI] = 1.379–5.860). The GSTM1 null genotype was more frequent in presbycusis subjects, i.e., 48 percent of control patients and 69 percent of white subjects carried this deletion (P = 0.0198, OR = 2.43, 95% CI = 1.163–5.067). The NAT2∗6A mutant genotype was more frequent among subjects with presbycusis (60%) than in control patients (34%; P = 0.0086, OR = 2.88, 95% CI = 1.355–6.141).CONCLUSIONWe showed an increased risk of presbycusis among white subjects carrying the GSTM1 and the GSTT1 null genotype and the NAT∗6A mutant allele. Subjects with the GSTT1 null genotypes are almost three times more likely to develop presbycusis than those with the wild type. The GSTM1 null genotype was more prevalent in white Hispanics than in white non‐Hispanics, but the GSTT1 and NAT2 polymorphisms were equally represented in the two groups.© 2010 American Academy of Otolaryngology‐Head and Neck Surgery Foundation. All rights reserved.

Plexiform neurofibromas are peripheral nerve sheath tumors associated with neurofibromatosis type 1. The maxillary sinus is an extremely rare location of the plexiform neurofibroma and only two adult cases have been previously reported. We report the first case of plexiform neurofibroma of the maxillary sinus occurring in a child with neurofibromatosis type 1. This unusual location presents a management challenge considering the infiltrative nature and the potential malignant degeneration of this type of tumor. MRI is highly valuable to diagnose and plan the surgical approach of the plexiform neurofibroma of the maxillary sinus. Due to the location of the tumor and the patient's age, conservative surgery is highly recommended. We performed an endoscopic total en-bloc resection of the tumor with no recurrence after nine months of follow-up.

Objective Identify prognostic factors affecting outcome in ossicular chain reconstruction (OCR). Methods Retrospective case series of electronic database at an academic institution. We reviewed 209 cases of OCR performed from January 2000 through September 2007, and systematically collected demographic, clinical, audiologic, and outcome information. Univariate analyses of group differences in terms of postoperative air‐bone gap (ABG) changes were evaluated by ANOVA. Multiple regression analyses were used to examine the relationship between postoperative air‐bone gap (dependent variable) and the independent variables. Results There were 107 cases of OCR which met inclusion criteria (45 primary and 62 revision tympanoplasties) with an average follow‐up of 19 months. There were 36 cases of chronic suppurative otitis media without cholesteatoma and 71 cases with cholesteatoma. The mean preoperative ABG was 32 ± 15 dB and the mean postoperative ABG was 23 ± 14 dB (P < 0.001). Of the independent variables analyzed, the type of procedure (i.e., tympanoplasty vs. canal‐wall up vs. canal‐wall down) and status of malleus handle were predictive of the success of OCR. Conclusions Favorable prognostic factors in OCR include smaller preoperative ABG and the presence of an intact malleus handle. Our findings support the practice of performing OCR at the time of the second‐look procedure.

Background. We reviewed management of the cervical lymph nodes in patients with Merkel cell carcinoma (MCC) of the head and neck.Methods. Records of 15 patients with MCC of the head and neck area were evaluated for the type of surgical treatment, including wide local excision, sentinel lymph node (SLN) biopsy, neck dissection, postoperative radiation therapy, and clinical outcomes.Results. Median follow-up was 24 months (range, 5-84 months). Ten patients were treated with wide local excision plus SLN, with or without neck dissection. Five patients were treated with wide local excision only or wide local excision plus neck dissection, One patient died of distant metastases (7%), and 14 patients remain alive (93%), over a mean follow-up of 24 months.Conclusion. Wide excision and SLN biopsy for primary MCC with NO neck is feasible for early-stage, previously untreated lesions. SLN biopsy was helpful in determining the nodal levels to be dissected or irradiated. (C) 2008 Wiley Periodicals, Inc. Head Neck 30: 1559-1565, 2008

The clinical utility of sentinel node biopsy for melanoma has led multiple investigators to apply this approach to other cutaneous malignancies as well as to early cancers of the upper aerodigestive tract. Data are most extensive for oral cancer. A multi-institutional pathologic validation trial for selected oral cancers provided negative predictive values of 96%. Subsequent trials should document clinical follow-up. This technique may ultimately play a wider role in the management of mucosal cancers.

We present the third case of mucoepidermoid carcinoma of the external auditory canal (EAC) in the English literature, and discuss the management of this lesion. The patient underwent a wide local resection, superficial parotidectomy, and selective neck dissection. Although intraoperative frozen section margins were negative, permanent histopathologica examination demonstrated tumor in the medial margin, and the tumor was upgraded to a high-grade mucoepidermoid carcinoma. The patient returned to the operating room for a wider local resection, and EAC reconstruction with a temporoparietal pedicled flap and split thickness skin graft. All margins were negative on final histopathologic examination. Radiotherapy was deferred in the event of a recurrence. The patient is currently disease-free 29 months after the final excision. Most authors advocate an aggressive surgical approach, which includes a form of a temporal bone resection, for the treatment of EAC carcinoma. Although this may be warranted in cases of squamous cell carcinoma, mucoepidermoid carcinoma of the EAC may be amenable to a conservative step-by-step approach for local control with less postoperative morbidity. Given the difficulty in detecting mucoepidermoid carcinoma in surgical margins by frozen section analysis, patients should be informed of the possibility of further surgery (re-resection) when a conservative approach is used.

The clinical utility of sentinel node biopsy techniques for cutaneous melanoma has led multiple investigators to study the applicability of this approach to other solid tumors, including cancers of the upper aerodigestive tract, and especially the oral cavity. Preliminary data indicate that it may be useful for early oral cancers, with the exception of floor of mouth tumors, where technical challenges related to the proximity of the lymphatic basin remain a problem. A multi-institutional pathologic validation trial, involving sentinel node biopsy followed by completion selective neck dissection, has completed accrual. While central step sectioning and immunohistochemistry remain to be completed and analyzed, routine pathologic techniques provided negative predictive values of 96% for oral cancer excluding floor of mouth lesions. Subsequent trials need to involve clinical follow-up and evaluation for recurrence in the neck. We believe this technique may ultimately play a role in the management of early oral cancer.

Objective: To report the surgical outcomes and safety of transnasal endoscopic resection (TER) for anterior skull base (ASB) tumors.Study Design and Setting: A retrospective chart review to identify patients undergoing TER for ASH tumors at a tertiary care medical center between September 1997 and June 2006.Results: Nineteen patients underwent TER for ASB tumors without open craniotomy. There were 17 malignant and two benign lesions. Olfactory neuroblastoma was the most common pathology, occurring in 53 percent of patients. One patient recurred locally, resulting in an overall local control rate of 94.7 percent for all neoplasms and 94.1 percent for malignant disease. It should be noted that the tumor control rate may be premature given the small sample size and limited follow-up. Overall, there were 16 complications, but only two of these, an orbital hematoma and a frontal lobe abscess, were considered major complications directly attributable to surgery.Conclusions: TER for ASB tumors appears to be safe in properly selected patients. In light of the small sample size and limited follow-up, the major complication rate directly attributable to surgery was I I percent, and the overall local control rate was 95 percent. A larger multi-institutional series with longer follow-up is warranted. (C) 2007 American Academy of Otolaryngology-Head and Neck Surgery Foundation. All rights reserved.

The clinical utility of sentinel node biopsy techniques for cutaneous melanoma has led multiple investigators to study the applicability of this approach to other solid tumors, including cancers of the upper aerodigestive tract, and especially the oral cavity. Preliminary data indicate that it may be useful for early oral cancers, with the exception of floor of mouth tumors, where technical challenges related to the proximity of the lymphatic basin remain a problem. A multi-institutional pathologic validation trial, involving sentinel node biopsy followed by completion selective neck dissection, has completed accrual. While central step sectioning and immunohistochemistry remain to be completed and analyzed, routine pathologic techniques provided negative predictive values of 96% for oral cancer excluding floor of mouth lesions. Subsequent trials need to involve clinical follow-up and evaluation for recurrence in the neck. We believe this technique may ultimately play a role in the management of early oral cancer.

Sentinel lymph node biopsy represents a less invasive technique for detecting subclinical lymphatic metastases in patients with a known primary malignancy. This procedure was developed to address the management of the lymphatics for cutaneous lesions, especially malignant melanoma. For melanoma, lymphatic drainage patterns are very unpredictable, and the therapeutic value of extensive formal lymphadenectomies remains controversial. The technique is increasingly being applied to other malignancies. Multiple small patient series have been published evaluating the application of lymphatic mapping and sentinel lymph node biopsy to cancer of the oral cavity. The technique requires the selection of patients without clinical or radiologic evidence of gross lymphatic cancer who are at risk for subclinical metastases. Subsequently, primary tumors are injected with a radioactive tracer, followed by radiologic imaging, and then gamma probe-guided lymph node excision through a small incision. Rigorous serial sectioning and immunohistochemistry is essential. Sentinel lymph node biopsy has not yet been validated as safe for oral cavity cancer, and a multi-institutional trial is currently completing accrual to correlate the histopathologic results of sentinel lymph node biopsy and subsequent selective neck dissection. The goal is to establish the predictive value of the less invasive procedure relative to formal lymphadenectomy. In this article, the authors describe the details of their surgical technique for sentinel lymphadenectomy as applied to oral squamous cell carcinomas. Sentinel lymph node biopsy represents a less invasive technique for detecting subclinical lymphatic metastases in patients with a known primary malignancy. This procedure was developed to address the management of the lymphatics for cutaneous lesions, especially malignant melanoma. For melanoma, lymphatic drainage patterns are very unpredictable, and the therapeutic value of extensive formal lymphadenectomies remains controversial. The technique is increasingly being applied to other malignancies. Multiple small patient series have been published evaluating the application of lymphatic mapping and sentinel lymph node biopsy to cancer of the oral cavity. The technique requires the selection of patients without clinical or radiologic evidence of gross lymphatic cancer who are at risk for subclinical metastases. Subsequently, primary tumors are injected with a radioactive tracer, followed by radiologic imaging, and then gamma probe-guided lymph node excision through a small incision. Rigorous serial sectioning and immunohistochemistry is essential. Sentinel lymph node biopsy has not yet been validated as safe for oral cavity cancer, and a multi-institutional trial is currently completing accrual to correlate the histopathologic results of sentinel lymph node biopsy and subsequent selective neck dissection. The goal is to establish the predictive value of the less invasive procedure relative to formal lymphadenectomy. In this article, the authors describe the details of their surgical technique for sentinel lymphadenectomy as applied to oral squamous cell carcinomas.