Publications by authors named "Spiros Manolidis"

56 Publications

Complications in Skull Base Surgery and Subsequent Repair.

Semin Plast Surg 2020 Nov 24;34(4):286-292. Epub 2020 Dec 24.

Otolaryngology and Facial Plastic Surgery Associates, Fort Worth, Texas.

Over the past several decades, endoscopic sinus surgery has revolutionized the approach to skull base surgery. Open skull base approaches remain a viable option for advanced skull base tumors. Complications have gone down with increased reliability of vascularized tissue transfer. In this article, the authors explore the various complications that can present following skull base surgery and review the approaches for repair when such issues are encountered.
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http://dx.doi.org/10.1055/s-0040-1721765DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7759428PMC
November 2020

Radiation Necrosis of the Lateral Skull Base and Temporal Bone.

Semin Plast Surg 2020 Nov 24;34(4):265-271. Epub 2020 Dec 24.

Neurotology, Head and Neck Surgery, Fort Worth, Texas.

Radiation therapy plays a critical role in the treatment of malignancies involving the head and neck. Although the therapeutic effects of ionizing radiation are achieved, normal tissues are also susceptible to injury and significant long-term sequelae. Osteoradionecrosis of the temporal bone (ORNTB) is among the many complications that can arise after therapy. ORNTB is a debilitating and potentially lethal condition that continues to challenge patients and treating physicians. Herein, we review the pathophysiology, presentation, work-up, and management of ORNTB.
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http://dx.doi.org/10.1055/s-0040-1721763DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7759429PMC
November 2020

Management of Skull Fractures and Calvarial Defects.

Facial Plast Surg 2019 Dec 29;35(6):651-656. Epub 2019 Nov 29.

Otolaryngology and Facial Plastic Surgery Associates, Fort Worth, Texas.

Scalp and calvarial defects can result from a myriad of causes including but not limited to trauma, infection, congenital malformations, neoplasm, and surgical management of tumors or other pathologies. While some small, nondisplaced fractures with minimal overlying skin injury can be managed conservatively, more extensive wounds will need surgical repair and closure. There are many autologous and alloplastic materials to aid in dural and calvarial reconstruction, but no ideal reconstructive method has yet emerged. Different reconstructive materials and methods are associated with different advantages, disadvantages, and complications that reconstructive surgeons should be aware of. Herein, we discuss different methods and materials for the surgical reconstruction of calvarial defects.
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http://dx.doi.org/10.1055/s-0039-3399522DOI Listing
December 2019

Rare Presentation of Giant Cell Tumor in the Internal Auditory Canal: Case Report and Review of the Literature.

J Neurol Surg Rep 2015 Jul 18;76(1):e65-71. Epub 2015 Feb 18.

Department of Otolaryngology, The Mount Sinai Medical Center Roosevelt Division, New York, New York, United States.

Giant cell tumor (GCT) is a benign but locally aggressive bone tumor that usually involves the end of long bones. It is a relatively common neoplasm in patients, constituting 5 to 10% of all benign bone tumors. Approximately 2% of GCTs occur in the craniofacial skeleton with a predilection for the ethmoid, sphenoid, and temporal bones. The skull base location is unique and not commonly described. Hearing loss, headache, tinnitus, and subcutaneous masses are the most commonly reported symptoms in GCTs of the skull base. In this case report we present the first description of a GCT within the internal auditory canal causing cranial neuropathy and review the recent pertinent literature.
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http://dx.doi.org/10.1055/s-0034-1396656DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4520973PMC
July 2015

DNA sequence analysis and genotype-phenotype assessment in 71 patients with syndromic hearing loss or auditory neuropathy.

BMJ Open 2015 May 19;5(5):e007506. Epub 2015 May 19.

Bobby R Alford Department of Otolaryngology-Head and Neck Surgery, Baylor College of Medicine, Houston, Texas, USA.

Objectives: Aetiological assessment of 71 probands whose clinical presentation suggested a genetic syndrome or auditory neuropathy.

Methods: Sanger sequencing was performed on DNA isolated from peripheral blood or lymphoblastoid cell lines. Genes were selected for sequencing based on each patient's clinical presentation and suspected diagnosis. Observed DNA sequence variations were assessed for pathogenicity by review of the scientific literature, and mutation and polymorphism databases, through the use of in silico tools including sorting intolerant from tolerant (SIFT) and polymorphism phenotyping (PolyPhen), and according to the recommendations of the American College of Medical Genetics and Genomics for the interpretation of DNA sequence variations. Novel DNA sequence variations were sought in controls.

Results: DNA sequencing of the coding and near-coding regions of genes relevant to each patient's clinical presentation revealed 37 sequence variations of known or uncertain pathogenicity in 9 genes from 25 patients. 14 novel sequence variations were discovered. Assessment of phenotypes revealed notable findings in 9 patients.

Conclusions: DNA sequencing in patients whose clinical presentation suggested a genetic syndrome or auditory neuropathy provided opportunities for aetiological assessment and more precise genetic counselling of patients and families. The failure to identify a genetic aetiology in many patients in this study highlights the extreme heterogeneity of genetic hearing loss, the incompleteness of current knowledge of aetiologies of hearing loss, and the limitations of conventional DNA sequencing strategies that evaluate only coding and near-coding segments of genes.
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http://dx.doi.org/10.1136/bmjopen-2014-007506DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4442153PMC
May 2015

External auditory canal defect management and reconstruction.

Dermatol Surg 2014 Sep;40 Suppl 9:S86-95

*Department of Otolaryngology and Head and Neck Surgery, Albert Einstein School of Medicine, Mount Sinai Roosevelt Hospital, Bronx, New York; †Department of Clinical Dermatology, Icahn School of Medicine, Mount Sinai, New York, New York; ‡Comprehensive Skin Cancer Center, Mount Sinai Beth Israel Medical Center and St. Luke's and Roosevelt Hospitals, New York, New York.

Background: The external auditory canal (EAC) is an area commonly involved by skin cancers. Knowledge of the anatomy of this area and proper evaluation and management of patients with these malignancies is essential.

Objective: The purpose of this article is to provide the reader with an understanding of the anatomy of the EAC, the options available for the treatment of EAC cancers, and repair of the resulting surgical defects.

Methods And Materials: A review of the current literature was performed to summarize the current literature on this topic.

Results: There are a variety of surgical options available for the treatment of these cancers whose use depends on the location and extent of the tumor. It is important to follow a logical reconstructive algorithm after tumor resection to optimize both functional and cosmetic results.
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http://dx.doi.org/10.1097/DSS.0000000000000118DOI Listing
September 2014

Initial experience with oropharynx-targeted radiation therapy for metastatic squamous cell carcinoma of unknown primary of the head and neck.

Anticancer Res 2014 Jan;34(1):243-8

Department of Radiation Oncology, Beth Israel Medical Center (BIMC), Continuum Cancer Centers of New York (CCCNY), 10 Union Square E. Suite 4 G, New York, NY 10003, U.S.A.

Aim: Metastasis of unknown primary (MUP) is commonly treated with radiation therapy (RT) to the entire mucosal surfaces and bilateral neck nodes (LN). We report outcomes of oropharynx-targeted RT, retropharyngeal nodes (RPN) and bilateral LN in this context.

Patients And Methods: Single-Institution retrospective study of 68 patients. Forty percent were treated with intensity-modulated radiation therapy (IMRT). Fifty-six percent received concurrent chemoradiotherapy (CCRT). The median age was 58 years, 82% were Caucasian, and 75% males. Stage III disease was present in 9%, stage IVA in 75% and IVB in 16%.

Results: At a median follow-up of 3.5 years, the actuarial locoregional control was 95.5%. The emergence of primary developed in 1patient (1.5%) and 2patients (3%) failed in the neck. The median time-to-locoregional failure (LRF) was 18 months. Actuarial long-term RT toxicity was grade 1 xerostomia (68%), dysphagia (35%), neck stiffness (15%) and trismus (6%).

Conclusion: RT to the oropharynx, RPN, and bilateral neck provides excellent oncological and functional outcomes in MUP in non-Asian patients. Sparing the mucosal surfaces of the nasopharynx, hypopharynx, and larynx seems reasonable without impacting on survival and locoregional control.
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January 2014

Long-term outcome of seropositive HIV patients with head and neck squamous cell carcinoma treated with radiation therapy and chemotherapy.

Anticancer Res 2013 Dec;33(12):5511-6

Department of Radiation Oncology, Beth Israel Medical Center (BIMC), Continuum Cancer Centers of New York (CCCNY), 10 Union Square E. Suite 4 G, New York, NY, 10003, U.S.A.

Aim: To report the outcome of radiation therapy (RT) +/- chemotherapy in HIV-seropositive patients with Head and Neck Squamous Cell Carcinoma (HNSCC).

Patients And Methods: This is the largest single-Institution retrospective study to date, consisting of 73 HIV patients with HNSCC treated from January 1997-2010. The median age at RT, HIV diagnosis and the duration of patients being HIV seropositive were 51, 34, and 11 years, respectively. Seventy patients had SCC and one had submandibular salivary duct carcinoma. Stages I-II, III and IVA/B were: 22%, 27% and 51%, respectively. Primary cancer sites comprised the larynx (37%), oropharynx (32%), oral cavity (13%), hypopharynx (7%), nasopharynx (4%), unknown primary (MUP) (4%), nasal cavity (3%), and submandibular salivary duct (1%). All patients had an ECOG performance scale of ≤1 and were treated with RT +/- chemotherapy. Fifty patients (70%) were on highly active anti-retroviral therapy (HAART) during treatment, and the median CD4 count was 290 (range: 203-1142). Median dose of 70, 63, and 54 Gy were delivered to the gross disease, high-risk neck, and low-risk neck respectively. Median duration of treatment was 52 (range: 49-64) days. Twelve patients (17%) underwent neck dissection for N3 disease.

Results: After a median follow-up of 47 months (range: 7-140), the 4-year locoregional control (LRC) and overall survival (OS) were 69% and 55% respectively. Seven patients (10%) developed second primary sites within the first 5 years of completing RT (2 anal SCCs and 5 HNSCCs). The LRC for Stages III/IV larynx and oropharynx SCC (which represent the majority of the cohort) were 76% and 70%, respectively. Chemo/RT-related late toxicities were dysphagia of grade≤2, 3, and 4 found in 74%, 15% and 11% of patients, respectively. Hoarseness (grade 1) was reported in 10% of patients; no patient experienced grade ≥2. Xerostomia grade ≤2, and 3 was found in 77% and 23% of patients, respectively. A Chi-square test and univariate analysis showed statistically significant relationships between LRC and duration of RT (p<0.001), as well as positive trends for weight loss (<10%) and absence of second malignancy.

Conclusion: Definitive RT +/- chemotherapy for HIV-seropositive patients with HNSCC appears to be less effective compared to the observed rates of LRC and OS of other HNSCC without HIV. Due to advances in the HAART which prolongs HIV patients' survival, it is extremely important to establish better treatment strategies to improve therapeutic ratio in this growing patient population.
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December 2013

An evolving paradigm for the workup and management of high-risk cutaneous squamous cell carcinoma.

J Am Acad Dermatol 2013 Oct 17;69(4):595-602.e1. Epub 2013 Jul 17.

Department of Dermatology, Division of Dermatologic Surgery, Columbia University Medical Center, New York, New York.

Background: No established standard of care exists for aggressive cutaneous squamous cell carcinoma (CSCC).

Objective: We sought to establish an aggressive CSCC management protocol by reviewing high-risk CSCC (HCSCC) and very high-risk CSCC (VCSCC) cases at our institution.

Methods: This was a retrospective review of all CSCC cases treated at our institution.

Results: A total of 27 patients were identified of 1591 cases treated between 2000 and 2011. Four patients with HCSCC received surgery alone and 1 received surgery and radiation. All remain disease free (median follow-up 5 years). Among patients with VCSCC, 4 received surgery alone: 1 (25%) showing a complete response and 3 (75%) showing disease progression. Eleven received surgery and radiation: 4 (36.4%) with complete response (median follow-up 3 years) and 7 (63.6%) with disease progression (median time to recurrence 6 months). Six received surgery and cetuximab: 3 (50%) had a complete response (median follow-up 3 years), 2 (33%) had disease progression, and 1 (14%) could not be assessed because of inability to tolerate infusions. One patient received surgery, cetuximab, and radiation, and remains disease-free after 4 years.

Limitations: Lack of randomization, blinding, a true control arm, or standardization of treatment protocols are limitations.

Conclusions: Patients with very HCSCC may have improved outcomes with surgery and adjuvant cetuximab.
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http://dx.doi.org/10.1016/j.jaad.2013.05.011DOI Listing
October 2013

Common cavity and custom-made electrodes: speech perception and audiological performance of children with common cavity implanted with a custom-made MED-EL electrode.

Int J Pediatr Otorhinolaryngol 2013 Aug 5;77(8):1237-43. Epub 2013 Jun 5.

Centro Clinico, Piazza Achille Leoni, 22, Rovereto, TN, Italy.

Objective: Few studies exist on children with common cavity, fewer still on their long-term audiological development after having received a cochlear implant. Our goal was to observe and report the long-term audiological progress of children with common cavity who were implanted with a custom-made electrode.

Methods: In this longitudinal, multi-center study, 19 children were implanted with a MED-EL custom-made electrode via either single slit cochleostomy or double posterior labyrinthotomy. We observed their audiological development with a test battery consisting of Categories of Auditory Performance (CAP), Speech Intelligibility Rating (SIR), and Ling 6-Sounds tests. We tested the children 1 month prior to the surgery; at first fitting; at 1, 3, 6, 12, and 18 months post first-fitting; at 2 years after first-fitting; and, whenever possible, at 3, 4, and 5 years after first-fitting.

Results: Children with common cavity tend to steadily and significantly improve their audiological skills over time. This development may, however, be highly individual; probably in part due to relatively high levels of additional needs. Parents should be counseled to establish realistic post-implantation expectations. Surgically, contrary to our expectations, we cannot confirm that double posterior labyrinthotomy reduces intracochlear electrode movement or that the MED-EL custom electrode leads to fewer incidences of intra- or post-implantation complications.

Conclusions: Cochlear implantation is a safe and effective treatment option in children with common cavity. The majority of children with CC derive significant audiological benefit from implantation. Intra- and post-surgical complications, while serious, and be dealt with effectively in most cases.
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http://dx.doi.org/10.1016/j.ijporl.2013.04.008DOI Listing
August 2013

Clinical validation and applications for CT-based atlas for contouring the lower cranial nerves for head and neck cancer radiation therapy.

Oral Oncol 2013 Sep 25;49(9):956-963. Epub 2013 Apr 25.

Department of Radiation Oncology, Beth Israel Medical Center, New York, NY, United States.

Objectives: Radiation induced cranial nerve palsy (RICNP) involving the lower cranial nerves (CNs) is a serious complication of head and neck radiotherapy (RT). Recommendations for delineating the lower CNs on RT planning studies do not exist. The aim of the current study is to develop a standardized methodology for contouring CNs IX-XII, which would help in establishing RT limiting doses for organs at risk (OAR).

Methods: Using anatomic texts, radiologic data, and guidance from experts in head and neck anatomy, we developed step-by-step instructions for delineating CNs IX-XII on computed tomography (CT) imaging. These structures were then contoured on five consecutive patients who underwent definitive RT for locally-advanced head and neck cancer (LAHNC). RT doses delivered to the lower CNs were calculated.

Results: We successfully developed a contouring atlas for CNs IX-XII. The median total dose to the planning target volume (PTV) was 70Gy (range: 66-70Gy). The median CN (IX-XI) and (XII) volumes were 10c.c (range: 8-12c.c) and 8c.c (range: 7-10c.c), respectively. The median V50, V60, V66, and V70 of the CN (IX-XI) and (XII) volumes were (85, 77, 71, 65) and (88, 80, 74, 64) respectively. The median maximal dose to the CN (IX-XI) and (XII) were 72Gy (range: 66-77) and 71Gy (range: 64-78), respectively.

Conclusions: We have generated simple instructions for delineating the lower CNs on RT planning imaging. Further analyses to explore the relationship between lower CN dosing and the risk of RICNP are recommended in order to establish limiting doses for these OARs.
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http://dx.doi.org/10.1016/j.oraloncology.2013.03.449DOI Listing
September 2013

Chondromyxoid fibroma of the mastoid portion of the temporal bone: MRI and PET/CT findings and their correlation with histology.

Ear Nose Throat J 2013 Apr-May;92(4-5):201-3

Department of Radiology, Beth Israel Medical Center, 10 Union Square East, New York, NY 10003, USA.

We report a very rare case of a chondromyxoid fibroma of the mastoid portion of the temporal bone in a 38-year-old woman who presented with left-sided hearing loss. Magnetic resonance imaging identified an expansile mass in the left mastoid bone with a heterogeneous hyperintense signal on T2-weighted imaging and peripheral enhancement. Subsequent positron emission tomography/computed tomography identified erosive bony changes associated with hypermetabolism. The patient underwent an infratemporal fossa resection with a suboccipital craniectomy/cranioplasty. We briefly review the aspects of this case, including a discussion of the differential diagnosis and the correlation between histologic and imaging findings.
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http://dx.doi.org/10.1177/014556131309200412DOI Listing
February 2014

Tolerance and toxicity of primary radiation therapy in the management of seropositive HIV patients with squamous cell carcinoma of the head and neck.

Laryngoscope 2013 May 26;123(5):1178-83. Epub 2013 Mar 26.

Department of Radiation Oncology, Beth Israel Medical Center, New York, New York, USA.

Objectives/hypothesis: To report tolerance and toxicity of radiotherapy (RT) with or without chemotherapy in HIV seropositive patients with squamous cell carcinoma of the head and neck (SCCHN).

Methods: This is a single institution retrospective study of 73 HIV seropositive patients with SCCHN treated from January 1997 through 2010. Stages I, II, III, and IV were 8%, 10%, 24%, and 58%, respectively. The median age at RT, HIV diagnosis. and the duration of HIV seropositive were 51 (32-72), 34 (25-50), and 11 (6-20) years, respectively. Patients were treated definitively with RT alone (35%) or concurrent chemo-RT (65%). Median dose of 70 Gy (66-70) was delivered to the gross disease. Median duration of treatment was 52 (49-64) days. Fifty patients (70%) were on HAART.

Results: RT± chemotherapy induced acute toxicity was: median weight loss 20 pounds (6-40), 100% developed dysgeusia and xerostomia (grades 1-3). Acute mucositis and dysphagia/odynophagia grades ≤ 2 and 3 were 83% and 17%, respectively. Treatment breaks in excess of 10, 7, and 3 days were found in 5%, 13%, and 15% of patients, respectively. With a median follow-up of 4 years (2-12) the RT ±chemotherapy induced late dysphagia and xerostomia grades >2 were 26% and 23% of patients, respectively.

Conclusion: Our data show that primary RT ±chemotherapy for HIV seropositive SCCHN is less tolerated compared to the historical data for SCCHN without HIV.

Level Of Evidence: 2b.
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http://dx.doi.org/10.1002/lary.23874DOI Listing
May 2013

Five-year outcomes of squamous cell carcinoma of the tonsil treated with radiotherapy.

Am J Clin Oncol 2014 Feb;37(1):57-62

Departments of *Radiation Oncology †Medical Oncology ‡Otolaryngology, Beth Israel Medical Center, New York, NY.

Purpose: To retrospectively review our single institution experience of patients with tonsillar squamous cell carcinoma.

Material And Methods: Between 1999 and 2005, a total of 79 patients were identified. Stage distribution was as follows: stages I-II, III, IVA, and IVB were in 6, 14, 43, and 16 patients, respectively. Sixty-three patients (80%) were male. Median age was 55.5 years. Treatment generally consisted of external beam radiation therapy (RT) (median dose, 70 Gy), concomitant chemotherapy (CCRT) (cisplatin 100 mg/m on days 1, 22, and 43), and neck dissection (ND), and was administered as follows: stages I/II, 6 patients received RT alone; stages III/IVA, 20, 5, and 32 patients received RT alone, CCRT, and CCRT followed by ND, respectively; stage IVB, 9 and 7 patients received CCRT and CCRT plus ND, respectively.

Results: After a median follow-up of 56 months (range, 12 to 122 mo), the 5-year local control (LC), regional control (RC), distant control (DC), and overall survival (OS) by stage were as follows: stage I-II 100%, 100%, 100%, 100%; stage III-IVA 98%, 96%, 95%, and 88%; stage IVB 100%, 100%, 69%, and 66%, respectively. Among stage IVB patients, DC was significantly lower (P=0.01) and a trend toward lower OS was noted (P=0.08). Long-term percutaneous endoscopic gastrostomy dependence was noted in 3% of them who had received CCRT. The effect of both chemotherapy and ND on treatment outcomes was analyzed; in stage III/IVA patients treated with or without chemotherapy, LC was 97% and 100% (P=0.43); RC was 92% and 100%(P=0.27); and DC was 91% and 94% (P=0.92), respectively. In stage III/IVA, patients treated with CCRT with or without ND, RC was 100% and 88%, respectively (P=0.087).

Conclusions: Primary radiotherapy with or without CCRT followed by ND provides excellent tumor control with acceptable toxicity in treating squamous cell carcinoma of the tonsil.
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http://dx.doi.org/10.1097/COC.0b013e31826b9920DOI Listing
February 2014

Trimodality management of sinonasal undifferentiated carcinoma and review of the literature.

Am J Clin Oncol 2013 Dec;36(6):584-8

*Department of Radiation Oncology ‡Department of Medical Oncology §Department of Otolaryngology, Beth Israel Medical Center, New York †Department of Radiation Oncology, Albert Einstein College of Medicine of Yeshiva University, Montefiore Medical Center, Bronx, NY.

Objective: Sinonasal undifferentiated carcinoma (SNUC) is a rare and aggressive malignancy with optimal management remains unclear. We performed a review of the impact of trimodality approach on SNUC outcome.

Methods: This is a single-institution retrospective study of 18 patients, who were managed between 1997 and 2009. The median age at presentation was 52 years (28 to 82). Nine patients (50%) were female. Three patients had stage II disease and underwent surgery alone, 12 had stages III and IVa and underwent surgery combined with chemoradiation, and 3 had stage IVb and underwent definitive chemoradiation. Patients who underwent preoperative, postoperative, and definitive chemoradiation received 60, 66, and 70 Gy of radiation, respectively. In all patients receiving concurrent chemoradiation, cisplatin was used, at a dose of 100 mg/m every 3 weeks for 3 cycles. Neoadjuvant chemotherapy included docetaxel, cisplatin, and 5-fluorouracil (TPF) every 3 weeks for 2 to 3 cycles.

Results: After a median follow-up of 26 months (16 to 120), a total of 8 patients (44%) have experienced the following: 1 persistent disease (5.5%), 4 local failure (22%), and 3 distant metastases (DM, 16.5%). Five of the 8 patients had preexisting cranial nerve deficits or gross cranial invasion. The 2-, 3-, and 4-year local control (LC), disease-free survival (DFS), and overall survival (OS) were 78%, 72%, and 56%; 75%, 65%, and 52%; and 75%, 50%, and 48%, respectively. Trimodality approach provided 83% LC and 92% DM-free survival, whereas other modalities provided 50% LC and 33% DM-free survival. The causes of death for the entire cohort were DM and local invasion. Acute chemoradiotherapy toxicity was 100% grades 1 and 2 dermatitis, mucositis, and fatigue, 55% developed grades 1 and 2 dysphagia, and 6% had grade 3 mucositis. Long-term toxicity was 28% grade 1 xerostomia, 11% retinopathy and optic neuropathy, and 6% orbital exenteration and grade 3 peripheral neuropathy.

Conclusions: SNUC is an aggressive neoplasm that frequently presents at an advanced stage. Our data show that trimodality approach in the form of surgery combined with chemoradiation seems to offer better LC and lower DM compared with other modalities.
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http://dx.doi.org/10.1097/COC.0b013e31825eb3a5DOI Listing
December 2013

Inroads toward robot-assisted cochlear implant surgery using steerable electrode arrays.

Otol Neurotol 2010 Oct;31(8):1199-206

Department of Mechanical Engineering, Columbia University, New York, New York, USA.

Hypothesis: Robotic insertions of actively steerable perimodiolar electrode arrays can substantially reduce insertion forces and prevent electrode buckling.

Background: Perimodiolar electrodes have been proven to be effective in reducing insertion forces. However, the dedicated techniques of atraumatic electrode insertion require intensive surgeon training. Although some specialized medical robots have been developed to help surgeons in certain minimally invasive surgeries, none are applicable to electrode insertions.

Methods: A robot prototype capable of automatically inserting novel steerable electrode array and adjusting its approach angle toward the scala tympani has been constructed and tested. Comparisons of insertion forces using robotically assisted steerable and straight electrodes on scala tympani models are presented. Simulations and experiments are conducted to compare the robotic insertion outcomes and insertion forces.

Results: The use of robotically assisted steerable electrodes for insertions significantly reduces the insertion forces compared with straight electrodes. Based on the results from the experiments, a second-generation robot with insertion force-sensing capability and haptic control to be used in the operating room has been designed for cochlear implant surgery.

Conclusion: Preliminary experimental results using robot-assisted steerable electrode prototype show that it is effective in reducing insertion forces and preventing electrode buckling. A second-generation robot has been designed and constructed for cochlear implant surgery under operating room conditions.
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http://dx.doi.org/10.1097/MAO.0b013e3181e7117eDOI Listing
October 2010

Basal cell carcinoma with intracranial invasion.

J Am Acad Dermatol 2009 Jun;60(6):1045-9

North Shore University Hospital at Plainview, Plainview, New York, USA.

The risk of invasion and destruction of cranium, underlying dura, and cranial nerves by basal cell carcinoma (BCC) is extremely low, with an estimated incidence of 0.03%. Intracranial BCC invasion by direct extension is rare, and orbital spread from a nasal lesion has not been reported in the literature. We describe a case of intracranial invasion of a multiply recurrent nasal BCC, which caused progressive bilateral blindness from optic nerve compression, with spinal canal involvement causing subsequent lower extremity weakness and paralysis. This case underscores the importance of early and appropriate treatment of high risk BCC, and aggressive treatment of recurrent lesions as early as possible.
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http://dx.doi.org/10.1016/j.jaad.2008.10.005DOI Listing
June 2009

Path planning and workspace determination for robot-assisted insertion of steerable electrode arrays for cochlear implant surgery.

Med Image Comput Comput Assist Interv 2008 ;11(Pt 2):692-700

Department of Mechanical Engineering, Columbia University Laboratory of Advanced Robotics and Mechanism Applications, RM 234, S.W., Mudd Building, 500 West 120th Street, New York, NY 10027, USA.

In previous works, the authors showed that using robot-assisted steerable electrode array insertions can significantly reduce the insertion forces compared to non-steerable electrode arrays. In addition to steering the electrode array, it is possible to change its angle of approach with respect to the scala tympani. This paper focuses on determining the relevance of changing the angle of approach of the electrode array by comparing steerable electrode array insertions using a two Degrees-of-Freedom (DoF) robot versus a four DoF robot. Optimal insertion path planning strategies are presented for both two and four DoF insertions. Simulation results and experiments show that the four DoF insertions can improve over two DoF insertions. Moreover, changing the angle of approach can further reduce the insertion forces. The simulation results also provide the workspace requirements for designing a custom parallel robot for robot-assisted cochlear implant surgery.
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http://dx.doi.org/10.1007/978-3-540-85990-1_83DOI Listing
December 2008

Intra-operative monitoring of cochlear function during cochlear implantation.

Cochlear Implants Int 2009 Mar;10(1):1-18

The Hearing Center at Texas Children's Hospital, Houston, Texas, USA.

The objective of this study was to determine if intra-operative auditory monitoring is feasible during cochlear implantation and whether this can be used as feedback to the surgeon to improve the preservation of residual hearing. This prospective non-randomised study was set in a paediatric tertiary referral hospital. Thirty eight consecutive paediatric patients undergoing cochlear implantation who had measurable auditory thresholds pre-operatively were divided into two cohorts. The unmonitored cohort included the first 22 patients and the monitored cohort included the last 16 patients. The main outcome measure(s) were pre-operative, intra-operative and more than one month post-operative average auditory thresholds at 500, 1000 and 2000 Hz measured using auditory steady-state response audiometry. The average pre-operative thresholds were 103.5 dB HL and 99.7 dB HL in the unmonitored and monitored cohorts, respectively. These were not statistically different (p > 0.3). In the monitored cohort, we measured auditory thresholds to assess cochlear function at multiple time points during the operation. Compared to baseline, thresholds were increased 0.7 dB after drilling the mastoidectomy and well, 0.2 dB after opening the cochlea and 4.6 dB after inserting the electrode array. One month post-operatively, the average thresholds were 114.0 dB HL in the unmonitored cohort but only 98.8 dB HL in the monitored cohort (p < 0.001). Both the use of intra-operative auditory monitoring and higher pre-operative thresholds were associated with improved preservation of residual hearing (p
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http://dx.doi.org/10.1002/cii.372DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3597106PMC
March 2009

Management of frontal sinus fractures.

Plast Reconstr Surg 2007 Dec;120(7 Suppl 2):32S-48S

New York, N.Y.; and Houston, Texas From the Department of Otolaryngology-Head and Neck Surgery, Beth Israel Hospital, and Baylor College of Medicine and Department of Plastic Surgery, Ben Taub General Hospital.

Frontal sinus fractures are relatively uncommon maxillofacial injuries, making up only 5 to 12 percent of all facial fractures. Associated intracranial, ophthalmologic, and other maxillofacial injuries are very common because of the force of injury required to fracture the frontal bone. High-resolution computed tomography of the frontal region in multiple planes is essential for predicting the degree of frontal injury, associated injuries, and the type of procedure indicated. Exploration of the frontal sinus with reduction alone is reserved for a small minority of very simple fractures. Most frontal sinus fractures will require the obliteration of the sinus. This is achieved in the majority of instances with preservation of the posterior wall. Those with more extensive injuries and the presence of a cerebrospinal fluid leak will require frontal sinus cranialization after repair of the dural injuries. In rare instances, primary bone grafts will be required. In both cranialization and obliteration procedures, the nasofrontal ducts must be managed appropriately to avoid complications. Newer techniques involving endoscopic image-guided surgery may offer an alternative for a small subset of patients with frontal sinus injury.
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http://dx.doi.org/10.1097/01.prs.0000260732.58496.1bDOI Listing
December 2007

Novel mutant-enriched sequencing identified high frequency of PIK3CA mutations in pharyngeal cancer.

Int J Cancer 2008 Mar;122(5):1189-94

The Department of Otolaryngology/Head an Neck Surgery, Columbia University College of Physicians and Surgeons, New York, NY 10032, USA.

We previously reported 4 PIK3CA mutations in 38 head and neck cancer samples, 3 of which were identified in 6 pharyngeal cancer samples. To determine the mutation frequency of PIK3CA in pharyngeal cancer, we studied 24 additional cases of pharyngeal squamous cell carcinoma in this study. Using both direct genomic DNA sequencing and novel mutant-enriched sequencing methods developed specifically for the 3 hot-spot mutations (H1047R, E545K and E452K) of PIK3CA, we detected 5 mutations of PIK3CA in the 24 pharyngeal cancers (20.8%). Three of the 5 mutations had been missed by the conventional sequencing method and were subsequently detected by novel mutant-enriched sequencing methods. We showed that the mutant-enriched sequencing method for the H1047R hot-spot mutation can identify the mutation in a mixed population of mutant and wild-type DNA sequences at 1:360 ratios. These novel mutant-enriched sequencing methods allow the detection of the PIK3CA hot-spot mutations in clinical specimens which often contain limited tumor tissues (i.e., biopsy specimens). The data further support that oncogenic PIK3CA may play a critical role in pharyngeal carcinogenesis, and the mutant-enriched sequencing methods for PIK3CA are sensitive and reliable ways to detect PIK3CA mutations in clinical samples. Because PIK3CA and its pathway are potential targets for chemotherapy and radiation therapy, and frequent somatic mutation of PIK3CA has been identified in many human cancer types (e.g., breast cancer, colorectal cancer), the abilities to detect PIK3CA mutations with enhanced sensitivities have great potential impacts on target therapies for many cancer types.
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http://dx.doi.org/10.1002/ijc.23217DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2792983PMC
March 2008

A pilot study of robot-assisted cochlear implant surgery using steerable electrode arrays.

Med Image Comput Comput Assist Interv 2006 ;9(Pt 1):33-40

Columbia University, Department of Mechanical Engineering, Laboratory of Advanced Robotics and Mechanism Applications, RM 234 S.W. Mudd Building, 500 West 120th Street, New York, NY 10027, USA.

This paper presents results of a pilot study evaluating the efficacy of robotic assistance using novel steerable electrode arrays for cochlear implant surgery. The current surgical setup of cochlear implant surgery is briefly reviewed and its limitations are highlighted. In an effort to reduce trauma to the structure of the cochlea, the kinematics and path planning for novel cochlear steerable electrodes are developed to minimize the interaction forces between the electrode and the cochlea. An experimental robotic system is used to compare the electrode insertion forces of steerable implants with those of nonsteerable electrodes. The results of these experiments show about 70% reduction in the insertion forces when steerable electrodes are used with our proposed path planning and control. A distance metric explaining this reduction in the insertion force is defined and experimentally validated. Although this is only a preliminary study, we believe that these results provide a strong indication to the potential of robot-assisted cochlear implant surgery to provide a significant reduction in trauma rates during cochlear implant surgery.
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http://dx.doi.org/10.1007/11866565_5DOI Listing
April 2007

Do corticosteroids prevent hearing loss in pediatric bacterial meningitis? An analysis of the evidence.

Ear Nose Throat J 2006 Sep;85(9):586-92

Department of Otolaryngology-Head and Neck Surgery, Columbia University College of Physicians and Surgeons, HP813, 180 Fort Washington, New York, NY 10032, USA.

We reviewed the MEDLINE database of articles published from January 1966 through December 2001 in search of data on the ability of the corticosteroid dexamethasone to protect against sensorineural hearing loss in children with meningitis. We found 1,034 articles that matched our keyword entries, and after various exclusions, we winnowed this number down to 16 articles that contained adequate data regarding audiometric evaluation and follow-up. The 16 articles included reports of 11 randomized controlled trials (only 10 are considered in this analysis), two meta-analyses, two retrospective case series, and two consensus statements. Of the 10 clinical trials (all of which contained level I evidence), four showed that dexamethasone had a protective effect and six showed that it did not. The authors of the two meta-analyses (both level I) concluded that there was a protective effect, and the authors of the two retrospective case series (both level IV) concluded that there was not. Both consensus statements (both level V) recommended the use of dexamethasone only in Haemophilus influenzae meningitis. We conclude that well-designed studies with level l evidence have shown that the benefit of dexamethasone in preventing hearing loss in children with meningitis remains unclear Significant variables in treatment response include the specific pathogen, the type of antibiotic, and the timing of dexamethasone administration.
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September 2006

DNA sequence analysis of GJB2, encoding connexin 26: observations from a population of hearing impaired cases and variable carrier rates, complex genotypes, and ethnic stratification of alleles among controls.

Am J Med Genet A 2006 Nov;140(22):2401-15

Bobby R. Alford Department of Otolaryngology-Head and Neck Surgery, Baylor College of Medicine, Houston, Texas 77030, USA.

Mutations in GJB2 are associated with hereditary hearing loss. DNA sequencing of GJB2 in a cohort of hearing impaired patients and a multi-ethnic control group is reported. Among 610 hearing impaired cases, 43 DNA sequence variations were identified in the coding region of GJB2 including 24 mutations, 8 polymorphisms, 3 unclassified variants (G4D, R127C, M163V), 1 controversial variant (V37I), and 7 novel variants (G12C, N14D, V63A, T86M, L132V, D159, 592_600delinsCAGTGTTCATGACATTC). Sixteen non-coding sequence variations were also identified among cases including the IVS1+1A>G mutation, 2 polymorphisms, and 13 novel variants. A diagnosis of GJB2-associated hearing loss was confirmed for 63 cases (10.3%). Heterozygous mutations were found in 39 cases (6.4%). Eleven cases carrying novel or unclassified variants (1.8 %) and 18 cases carrying the controversial V37I variant were identified (3%). In addition, 294 control subjects from 4 ethnic groups were sequenced for GJB2. Thirteen sequence variations in the coding region of GJB2 were identified among controls including 2 mutations, 6 polymorphisms, 2 unclassified variants (G4D, T123N), 1 controversial variant (V37I), and 2 novel variants (R127L, V207L). Nine sequence variations were identified among controls in the non-coding regions in and around GJB2 exon 2. Of particular interest among controls were the variability in carrier rates and ethnic stratification of alleles, and the complex genotypes among Asians, 47% of whom carried two to four sequence variations in the coding region of GJB2. These data provide new information about carrier rates for GJB2-based hearing loss in various ethnic groups and contribute to evaluation of the pathogenicity of the controversial V37I variant.
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http://dx.doi.org/10.1002/ajmg.a.31525DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3623690PMC
November 2006

Clinical predictors for hearing loss in children with bacterial meningitis.

Arch Otolaryngol Head Neck Surg 2006 Sep;132(9):941-5

Bobby R. Alford Department of Otolaryngology-Head and Neck Surgery, Baylor College of Medicine, Houston, Tex, USA.

Objectives: To identify clinical risk factors that predict a higher incidence of hearing loss in children with bacterial meningitis, to determine the overall incidence of hearing loss in a large group of children proven by culture findings to have bacterial meningitis, and to compare clinical characteristics among patients with Streptococcus pneumoniae meningitis and Neisseria meningitidis meningitis.

Design: Retrospective review

Setting: Tertiary pediatric hospital.

Patients: A total of 171 children identified with bacterial meningitis who met inclusion criteria over a consecutive 10-year period.

Main Outcome Measure: Presence of sensorineural hearing loss.

Results: Of 134 patients who underwent audiologic testing during their initial hospitalization, 41 (30.6%) were found to have at least a unilateral mild sensorineural hearing loss. The incidence of hearing loss was greater in patients with S pneumoniae meningitis than in patients with N meningitidis meningitis (35.9% and 23.9%, respectively). Length of hospitalization, development of seizures, elevated cerebrospinal fluid protein, and decreased cerebrospinal fluid glucose were significant predictors for hearing loss in children with bacterial meningitis. These factors were not found to be as strong a predictor for hearing loss in patients with N meningitidis meningitis. Stability of hearing was demonstrated with limited follow-up audiometry.

Conclusions: Sensorineural hearing loss is a common sequela in children with bacterial meningitis. Identification of hearing loss in children with bacterial meningitis and early rehabilitation will lessen the long-term educational and social difficulties these children may experience.
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http://dx.doi.org/10.1001/archotol.132.9.941DOI Listing
September 2006

Single-stage total and near-total resection of massive pediatric head and neck neurofibromas.

J Craniofac Surg 2006 May;17(3):506-10

Otolaryngology-Head & Neck Surgery, Columbia University, New York, New York, USA.

Plexiform neurofibromas traditionally have posed a surgical challenge in pediatric patients. Expert preoperative planning, advanced surgical techniques, and vigilant postoperative care results in minimal morbidity and resolution of tumor symptomatology. A retrospective review of four consecutive pediatric patients with massive head and neck neurofibromas who underwent single-stage total or near-total removal of their tumors was performed. All four patients had obstructive airway symptoms. There were two minor complications. All patients had complete relief of their symptoms and return of function without any additional neurologic deficits. It is possible to safely achieve total or near-total removal of extensive plexiform neurofibromas with minimal morbidity while restoring lost function.
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http://dx.doi.org/10.1097/00001665-200605000-00020DOI Listing
May 2006

Cochlear nerve size evaluation in children with sensorineural hearing loss by high-resolution magnetic resonance imaging.

Am J Otolaryngol 2006 May-Jun;27(3):166-72

Department of Otolaryngology, Texas Children Hospital, Houston, TX, USA.

Purpose: To determine differences in size of cochlear nerves among subjects with deafness due to connexin 26 (Cx26) mutations, subjects with deafness of unknown origin, and normal hearing subjects by sagittal high-resolution magnetic resonance (HRMR) imaging of the temporal bone.

Materials And Methods: Cross-sectional and surface areas and volumetric measurements of the cochlear nerve and modiolus were made on HRMR images of the internal auditory canal (IAC) and inner ear in the 3 groups of children (groups 1, 2, and 3). Three-way comparisons of in vivo cochlear nerve measurements on HRMR imaging were made among 17 children with sensorineural hearing loss (SNHL) and no obvious etiology for the hearing loss (group 1), 7 children with profound SNHL due to a Cx26 mutation (group 2), and 10 normal hearing children (group 3).

Results: Children with profound SNHL of unknown cause and children with profound SNHL due to a connexin mutation displayed hypoplastic cochlear nerves as compared with normal controls. HRMR imaging of the temporal bone was accurately delineated potential problems with cochlear nerves in 2 of 17 instances where high-resolution computed tomography did not do so.

Conclusions: Accurate and specific measurements of the cochlear nerve and related structures is possible on HRMR imaging of the temporal bone. The size of the cochlear nerve is mildly hypoplastic in children with profound SNHL of unknown causes or children with a deafness-causing Cx26 mutation. HRMR imaging is superior to high-resolution computed tomography in the investigation of profound SNHL in children.
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http://dx.doi.org/10.1016/j.amjoto.2005.09.007DOI Listing
October 2006

PIK3CA mutations in head and neck squamous cell carcinoma.

Clin Cancer Res 2006 Mar;12(5):1441-6

Department of Otolaryngology/Head and Neck Surgery, Columbia University College of Physicians and Surgeons, New York, New York 10032, USA.

Purpose: Recent studies have reported high frequencies of somatic mutations in the phosphoinositide-3-kinase catalytic alpha (PIK3CA) gene in several human solid tumors. Although gene amplifications of PIK3CA have been reported in head and neck squamous cell carcinoma (HNSCC), small mutation of the gene has not been evaluated in HNSCC previously. In this study, we examined the mutation frequency of PIK3CA in HNSCC.

Experimental Design: More than 75% of the somatic mutations of PIK3CA are clustered in the helical (exon 9) and kinase domains (exon 20). To investigate the possible role of PIK3CA in HNSCC tumorigenesis, exons 1, 4, 5, 6, 7, 9, and 20 of the gene were analyzed by direct genomic DNA sequencing in 38 HNSCC specimens.

Results: We identified four missense mutations in the seven exons of PIK3CA from 38 HNSCC specimens (11%). Three of the four mutations (i.e., H1047R, E542K, and E545K) have been previously reported as hotspot mutations. The remaining novel mutation, Y343C, is identified at exon 4 nucleotide 1028 A --> G. Three of the four mutations were shown to be somatic, whereas the fourth mutation (H1047R) was identified in a cell line. Interestingly, three of the four mutations identified were in pharyngeal cancer samples.

Conclusions: These data provide evidence that oncogenic properties of PIK3CA contribute to the carcinogenesis of human head and neck cancers, especially in pharyngeal cancer. A specific kinase inhibitor to PIK3CA may potentially be an effective therapeutic reagent against HNSCC or pharyngeal cancer in particular.
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http://dx.doi.org/10.1158/1078-0432.CCR-05-2173DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1780023PMC
March 2006

Endoscopically guided placement of prefabricated cochlear implant electrodes in a common cavity malformation.

Int J Pediatr Otorhinolaryngol 2006 Apr 7;70(4):591-6. Epub 2006 Feb 7.

Department of Otolaryngology-Head and Neck Surgery, Columbia University, 180 Fort Washington Street, HP812, New York, NY 10032, USA.

Objective: To devise a safe and effective method of optimal customized electrode placement in the common cavity of children with cochleovestibular malformations.

Methods: Specialized electrodes were manufactured on the basis of three-dimensional data obtained from the high resolution computed tomography (HRCT) scans of the temporal bones of these two children. Electrode positioning was achieved with direct endoscopic view of the cavity utilizing a three-hole common cavity technique.

Results: Optimal electrode positioning in apposition to the medial neuroepithelium in the common cavity was verified visually intraoperatively. Postoperatively, minimal stable electrical current levels were found to be required.

Conclusions: Custom-designed electrodes have the potential to offer improved results in children with common cavity malformations. Intraoperative direct positioning may further improve these results.
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http://dx.doi.org/10.1016/j.ijporl.2005.07.004DOI Listing
April 2006