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Objectives. As a result of universal newborn hearing screening and improved evaluation tools, many children with severe to profound hearing loss are being diagnosed as infants. This affords the opportunity to provide these children access to cochlear implantation, although medical and audiologic challenges must be addressed. The purpose of this study was to investigate the safety and efficacy of cochlear implantation in children who are younger than 1 year. Methods. A prospective study was conducted of 18 children who had confirmed severe to profound sensorineural hearing loss and received cochlear implants at our medical center before 12 months of age. The length of device usage ranged from 6 months to 4 years, 5 months. The main outcomes measured were perioperative and postoperative surgical/medical aspects, the Infant-Toddler Meaningful Auditory Integration Scale and age-appropriate phoneme, and word and sentence recognition tests, when appropriate. Results. All children had full insertions of the electrode array without surgical complications and are developing age-appropriate auditory perception and oral language skills. Conclusions. Early implantation is feasible and beneficial in some children who are younger than 12 months and should be considered with attention to variables involved in the decision-making process, including possible increased surgical risk, skull size and scalp thickness, and mastoid development.

Facial nerve outcomes and surgical complication rates for other cranial nerves were evaluated retrospectively after the resection of large acoustic neuromas. The charts of all patients who underwent surgical removal of an acoustic neuroma between 1992 and 2001 at New York University Medical Center were reviewed. Fifty-four patients with tumors measuring 3 cm or larger were included in the study. Four patients had neurofibromatosis type 2, two of whom underwent bilateral removal of acoustic neuromas. Translabyrinthine microsurgical removal of tumor was performed in 47 of 56 cases (84%). In all cases, EMG monitoring, improved sharp microdissection, and ultrasonic aspiration were employed. Facial nerve function was assessed using the House-Brackmann facial nerve grading system immediately after surgery and at follow-up visits. A House-Brackmann grade III or better was achieved in 90% of patients, and a grade II or better was achieved in 84% of patients. Ultimate facial nerve outcome was excellent after the surgical resection of large acoustic neuromas. Preoperative cranial nerve palsies also improved after surgery. The translabyrinthine approach for tumor removal is our treatment of choice for acoustic neuromas 3 cm or larger.

Problem: Endolymphatic sac tumor (ELST) is a rare vascular neoplasm associated with von Hippel-Landau disease, an autosomal dominant multitumor syndrome characterized by highly vascular tumors. The von Hippel-Landau (VHL) gene is located on chromosome 3p25 and encodes the VHL tumor suppressor protein, which plays a critical role in the degradation of HIF-1a. HIF-1a regulates vascular endothelial growth factor (VEGF)-mediated angiogenesis. The lack of degradation of HIF-1a due to a nonfunctioning VHL protein is thought to play a role in the proliferation of tumors associated with VHL syndrome. The goals of this study are to present clinical and radiological presentations of 3 patients with ELSTs and report on the HIF-1a overexpression in ELST cells. Methods: Our study consisted of chart and radiological review and immunohistochemical analysis. Specimens from 3 patients with pathologically confirmed ELST were analyzed for the presence of the HIF-1a protein using imunohistochemistry with a specific monoclonal antibody as previously described (Zagzag et al, Cancer 2000). Results: All patients presented with nonspecific findings seen with other temporal bone-related lesions of ipsilateral tinnitus and hearing loss. One patient was evaluated at a late stage who also presented with facial nerve weakness. Immunohistochemical analysis in all specimens confirmed overexpression of the HIF-1a protein in tumor cells. Conclusion: ELSTs are likely to be associated with HIF-1a mediated angiogenesis. These lesions are possibly associated with loss of VHL protein due to mutation of the VHL gene. Significance: Elucidation of the pathophysiology of endolymphatic sac tumors may lead to future novel approaches in the management of this unusual tumor associated with von Hippel-Lindau disease. Support: None reported.

Objectives: Patients who require surgery for chronic otitis media with perforation and cholesteatoma frequently have scarce residual tympanic membrane or cholesteatoma adherently involving its entire undersurface. These situations prove poor for standard underlay tympanoplasty techniques. A novel technique is described that maximizes healing and minimizes complications of mucosalization and anterior blunting. The results are compared using Alloderm vs temporalis fascia as grafting materials. Fascia is used when available in primary cases, while AlloDerm has proven successful in revision cases or when delayed epithelialization can be expected. Methods: A retrospective review was performed on all otologic cases at a tertiary care public hospital from 1999 to 2003. Fifty-five cases where total reconstruction was required were included in the study. Simple underlay grafting cases were excluded. Data reviewed included preoperative diagnosis, intraoperative findings, grafting material used, surgical technique, postoperative healing, complications. The group of patients who underwent reconstruction using fascia were compared to those who underwent AlloDerm reconstruction. Results: The 2 groups of patients were similar in preoperative diagnosis, intraoperative findings, surgical technique, and postoperative healing. The AlloDerm group had a slightly higher postoperative perforation rate, likely due to pores within the AlloDerm itself. The closure rate in the Alloderm group was still high at 93%. Audiologic data is analyzed. Conclusions: Temporalis fascia and AlloDerm are both safe and highly effective grafting materials in the reconstruction of the total tympanic membrane perforation if used with the described technique.

In recent years, new designs of cochlear implant electrodes have been introduced in an attempt to improve efficiency and performance by locating stimulation sites closer to spiral ganglion neurons and deeper into the scala tympani. The goal of this study was to document insertion depth, intracochlear position and insertion trauma with the Nucleus Contour electrode and to compare results to those observed with the earlier generation Nucleus banded electrode. For this comparison eight Nuclears banded electrodes and 18 Contour electrodes were implanted in cadaver temporal bones using a realistic surgical exposure. Two experienced cochlear implant surgeons and two otology fellows with specialized training in cochlear implant surgery were selected for the study to represent a range of surgical experience similar to that of surgeons currently performing the procedure throughout the world. Following insertion of the electrodes, specimens were imaged using plain film X-ray, embedded in acrylic resin, cut in radial sections with the electrodes in place, and each cut surface was polished. Insertion depth was measured in digitized X-ray images, and trauma was assessed in each cross-section. The Contour electrode inserted more deeply (mean depth=17.9 mm or 417 degrees ) than the banded electrode (mean depth=15.3 mm or 285 degrees ). The incidence and severity of trauma varied substantially among the temporal bones studied. However, the nature and frequency of injuries observed with the two devices were very similar. The Contour electrode was clearly positioned closer to the modiolus than the banded model, and also appeared easier to use. Based on this difference in position and data from previous studies we conclude that the Contour electrode may provide lower thresholds and improved channel selectivity, but the incidence of trauma remains a problem with the newer design. The relative influences of electrode positioning and neural degeneration that may result from trauma are as yet unclear.

Objective To evaluate the efficacy of cochlear implants in human immunodeficiency virus-infected individuals and correlate these results with a proposed pathophysiological mechanism of human immunodeficiency virus-associated hearing loss. Study design Retrospective case series and temporal bone analysis of deceased human immunodeficiency virus-positive patients. Setting Tertiary care hospital. Patients Seven human immunodeficiency virus-positive individuals with profound sensorineural hearing loss. Intervention Cochlear implantation at New York University Medical Center. Methods The surgical outcomes and complications were analyzed. Additionally, electron microscopic and immunohistochemical findings of cadaver temporal bone specimens of other known human immunodeficiency virus-positive individuals were reviewed. The performance results of the human immunodeficiency virus-positive cochlear implant patients were then correlated with the previously hypothesized pathophysiological mechanism of human immunodeficiency virus-associated hearing loss. Results The patients had a varied performance with cochlear implantation, and as a group performance was good. There were no surgical complications or postoperative complications. The good performance of these patients supports the hypothesis that the mechanism of human immunodeficiency virus-associated deafness involves infiltration, malfunction, and premature degeneration of the hair cells and supportive cells of the cochlea. Conclusions Human immunodeficiency virus-positive individuals benefit from cochlear implantation without increased surgical risk.

Motion sickness can be a limiting factor for sea and air missions. We report the experience of a Pararescue (PJ) team on a Pacific Ocean rescue mission in which motion sickness was prevalent. Cinnarizine, an antagonist of H1-histamine receptors, was used to treat affected PJs. We also report findings of a survey of PJs regarding motion sickness. A family of four on a disabled sailboat 900 miles off the coast of Mexico sent out a distress call because their 1-year-old daughter became severely ill with fever and diarrhea. Four PJs were deployed on a C-130, performed a free-fall parachute insertion into the ocean, and boarded the sailboat. All four PJs experienced onset of motion sickness at some point during the early part of the mission and symptoms persisted through the first 24 hours. Three PJs experienced ongoing nausea, vomiting, dizziness, and sensory imbalances. The captain of the sailboat offered the three sick PJs approximately 18mg of cinnarizine two or three times a day with relief of symptoms and improvement on operational effectiveness. A new, anonymous, voluntary survey of Air National Guard PJs and combat rescue officers revealed that 78.4% of Operators have experienced motion sickness at sea. We discuss the current theories on motion sickness, the effect of motion sickness on operational effectiveness, and research on treatment of motion sickness, including the medication cinnarizine.

This work presents measurements of the parity-violation parameters A{sub c} and A{sub b} made at the Z pole. These measurements include the data taken with the SLD detector at the SLAC Linear Collider (SLC) during the period 1996-98. Heavy flavor events are selected with high efficiency and purity by searching for displaced vertices, identified with the SLD precision CCD vertex detector. Two methods are used for quark/antiquark discrimination: the net charge of the displaced vertex, and tracks in the displaced vertex identified as kaons by the SLD Cherenkov Ring Imaging Detector (CRID). The signal purities and analyzing powers are calibrated from the data to reduce the systematic errors and avoid experimental bias. The results are A{sub c} = 0.673 {+-} 0.029 {+-} 0.023 and A{sub b} = 0.919 {+-} 0.018 {+-} 0.017, where the first error is statistical and the second systematic. Fits to the electroweak data performed by the LEP Electroweak Working Group are used to study the consistency of the Standard Model, and to constrain the mass of the Standard Model Higgs boson.

Objective: Since 1987, when the use of multichannel cochlear implants was initiated in children, candidacy has expanded; many thousands of children have received these devices, and results have revealed a wide range of performance. However, few long-term studies exist on a large population of these children. There have been concerns expressed that cochlear implant function might degrade over time, that devices and electrodes might migrate and extrude in the growing child, or that there might even be a deleterious effect of long-term stimulation of the cochlear nerve. The purpose of this study was to explore the long-term effects of implantation as a function of performance over time, reimplantation, and educational factors. Study Design and Setting: We studied 81 children who received implants at a major academic medical center and were followed for 5 to 13 years. Results: Results revealed significant gains in speech perception, use of oral language, and ability to function in a mainstream environment. There was no decrease in performance over time and no significant incidence of device or electrode migration or extrusion, and device failure did not cause a deterioration in long-term outcome. Conclusions: Multichannel cochlear implants in children provide perception, linguistic, and educational advantages, which are not adversely affected by long-term electrical stimulation. (Otolaryngol Head Neck Surg 2002;126:505-11.)

To determine the efficacy of cochlear implants with current processing strategies in children and adults with long-term congenital deafness, and to determine whether the growth of auditory perceptual skills in these patients is similar to the data reported for patients who have undergone implantation with earlier devices and coding strategies.A prospective study of children and adults fulfilling the study inclusion criteria.University medical center.Thirty-five congenitally deaf children who received implants after the age of 8 years and 14 congenitally deaf adults who received implants as adults participated in this study. Length of device use ranged from 6 months to 3 years.Open set phoneme, word recognition tests, and sentence recognition tests were administered in quiet and noise auditory only conditions preoperatively and postoperatively. Changes in test scores were examined by paired t tests, and differences between groups were compared by Student's t tests. Spearman correlation coefficients were calculated to identify associations between scores and subject characteristics. A two-sided alpha of less than 0.05 was considered statistically significant.The results indicated significant improvement in open set speech perception skills in the children after implantation; the adults demonstrated improved mean scores on both word and sentence recognition. A shorter length of deafness correlated with better postoperative performance, but all subjects continued to improve over time. The improvement in test scores was similar between the devices, and no significant differences were detected between the different processing strategies.Children and adults with long-term congenital deafness can obtain considerable open set speech understanding after implantation. Length of deafness (age at implantation), length of device use, and mode of communication contribute to outcome.

BACKGROUND: Vestibular schwannomas (VS) and meningiomas are associated with biallelic loss of NF2, and may arise sporadically or in the context of the tumor predisposition syndrome neurofibromatosis type 2. Loss of NF2 activates the mammalian target of rapamycin (mTOR) pathway, and published in vitro and in vivo data indicate that mTOR inhibition may be effective in the treatment of NF2-deficient VS and meningiomas. Everolimus is an oral inhibitor of mTOR complex 1 (mTORC1) with anti-tumor activity in a variety of tumors. METHODS: We conducted a retrospective review of patients with NF2 and progressive vestibular schwannomas treated with everolimus on our prospective phase II clinical trial (ClinicalTrials.gov NCT01419639). Everolimus was administered at a daily dose of 10 mg in continuous 28-day courses for up to 12 courses. In this retrospective study, we included patients with at least one volumetrically measurable meningioma (>0.5 cc) who received at least six 28-day courses of therapy. Tumor response was assessed with brain MRI every three months using three-dimensional volumetric analysis. RESULTS: Three patients met criteria and had 10 evaluable meningiomas, with a total combined volume of 11.84 cc at baseline (median 1.20 cc, range 0.53–3.00 cc). Median time on therapy was 9 months (range 6–12 months). Total combined meningioma volume remained stable during treatment (-1.8%). Correspondingly, none of the individual tumors analyzed met criteria for volumetric response or progression during the treatment period (range -10% to +9.4%). CONCLUSIONS: Neither objective response nor progression was appreciated in meningiomas patients during a median treatment duration of 6 months. Further clinical studies will be required to determine whether everolimus affects meningioma growth velocity in patients with NF2. To better understand pharmacologic mTOR signaling pathway modulation in human VS and meningiomas in vivo, we are currently conducting a multi-center pharmacodynamic/pharmacokinetic (“phase 0”) study with everolimus (NCT01880749).

This study was conducted to evaluate the insertion properties and intracochlear trajectories of three perimodiolar electrode array designs and to compare these designs with the standard Cochlear/Melbourne array.Advantages to be expected of a perimodiolar electrode array include both a reduction in stimulus thresholds and an increase in dynamic range, resulting in a more localized stimulation pattern of the spiral ganglion cells, reduced power consumption, and, therefore, longer speech processor battery life.The test arrays were implanted into human temporal bones. Image analysis was performed on a radiograph taken after the insertion. The cochleas were then histologically processed with the electrode array in situ, and the resulting sections were subsequently assessed for position of the electrode array as well as insertion-related intracochlear damage.All perimodiolar electrode arrays were inserted deeper and showed trajectories that were generally closer to the modiolus compared with the standard electrode array. However, although the precurved array designs did not show significant insertion trauma, the method of insertion needed improvement. After insertion of the straight electrode array with positioner, signs of severe insertion trauma in the majority of implanted cochleas were found.Although it was possible to position the electrode arrays close to the modiolus, none of the three perimodiolar designs investigated fulfilled satisfactorily all three criteria of being easy, safe, and atraumatic to implant.

Objective: To determine cochlear implant electrode stability in the young patient. Electrode migration due to future skull growth was a concern that led to prohibiting implantation in children less than 2 years of age. Recently, the high level of performance achieved by young implantees has led to a re-evaluation of this lower age limit, requiring an assessment of the effects of skull growth over time. Study Design: Prospective radiographic analysis of electrode position of cochlear implants in young children. Methods: Twenty-seven children implanted with the Nucleus (Cochlear Corp., Denver, CO) or Clarion (Advanced Bionics Inc., Sylmar, CA) multichannel cochlear prostheses were subjects for this study. Follow-up radiographic studies were obtained for a period of month to 5 years after implantation. The age at time of implantation ranged from 14 months to 5 years. An intraoperative modified Stenver's view plain radiograph was obtained as a baseline. After implantation, on a yearly basis transorbital Stenver's and base views were obtained for comparative purposes. Additional radiographs were obtained whenever a change in performance or electrode map caused suspicion for extrusion. Electrode position was determined using a computer graphics enhancement technique whereby image contrast filters enhanced the visibility of the electrode array and surrounding bony structures. Results: An analysis of the data revealed no migration of the electrode array over time. Conclusions: The confirmation of the stability of the electrode array alleviates the concern of the effects of skull growth on cochlear implantation in young children.

Objective: Facial nerve paralysis or compromise can be caused by lesions of the temporal bone and cerebellopontine angle and their treatment. When the facial nerve is transected or severely compromised and primary end-to-end repair is not possible, hypoglossal-facial nerve anastomosis remains the most popular method for accomplishing three main goals: restoring facial tone, restoring facial symmetry, and facilitating return of voluntary facial movement. Our objectives are to evaluate the surgical feasibility and long-term outcomes of our technique of direct facial-to-hypoglossal neurorrhaphy with a parotid-release maneuver. Design: Prospective cohort. Setting: Academic tertiary care referral center. Patients: Ten patients with facial paralysis from proximal nerve injury underwent the facial-hypoglossal neurorrhaphy with a parotid-release maneuver. Main outcome measures: The Repaired Facial Nerve Recovery Scale, questionnaires, and photographs. Results: Facial-hypoglossal neurorrhaphy with parotid release was technically feasible in all cases, and anastomosis was performed distal to the origin of the ansa hypoglossi. All patients had good return of facial nerve function. Nine patients had scores of C or better, indicating strong eyelid and oral sphincter closure and mass motion. There was no hemilingual atrophy and no subjective tongue dysfunction. Conclusions: The parotid-release maneuver mobilizes additional length to the facial nerve, facilitating a tensionless communication distal to the ansa hypoglossi. The technique is a viable option for facial reanimation, and our patients achieved good clinical outcomes with continual improvement.

A retrospective analysis was performed on 174 patients operated on from 1992 to 1995 to evaluate the safety and efficacy of continuous lumbar cerebrospinal fluid drainage (CLCFD) in the management of cerebrospinal fluid (CSF) leaks following acoustic neuroma surgery. There was a 17% incidence of CSF leak. CLCFD stopped the leak in 87% of cases. There were no cases of meningitis associated with CLCFD. One deep vein thrombosis was treated without sequelae. It is concluded that CLCFD is safe and efficacious. The authors recommend that CLCFD be implemented immediately when indicated, foregoing a trial of conservative therapy. Indications and precautions are discussed.

Objectives To determine the efficacy of cochlear implantation (CI) in prelingually deafened adolescent children and to evaluate predictive variables for successful outcomes. Design Retrospective medical record review. Participants Children aged 10 to 17 years with prelingual hearing loss (mean length of deafness, 11.5 years) who received a unilateral CI (mean age at CI, 12.9 years). Intervention Unilateral CI. Main Outcome Measures Standard speech perception testing (Consonant-Nucleus-Consonant [CNC] monosyllabic word test and Hearing in Noise [HINT] sentence test) was performed preoperatively, 1 year postoperatively (year 1), and at the last follow-up/end of the study (EOS). Results There was a highly significant improvement in speech perception scores for both HINT sentence and CNC word testing from the preoperative testing to year 1 (mean change score, 51.10% and 32.23%, respectively; P Conclusions Adolescents with prelingual deafness undergoing unilateral CI show significant improvement in objective hearing outcome measures. Patients with shorter lengths of deafness and earlier age at CI tend to outperform their peers. In addition, patients with progressive deafness and those using oral communication have significantly better objective outcomes than their peers.

Eight patients 45 years of age and under (range, 29 to 45) with squamous cell carcinoma of the head and neck and infection with the human immunodeficiency virus are reported. Primary tumor sites include nasopharynx, oral cavity, oropharynx, and larynx. Probes for the human papillomavirus were positive in two of the patients. Therapy consisted of surgery followed by radiation therapy in five patients, surgery alone in one patient, and radiation therapy in the remaining two patients. Follow-up ranged up to 2 years and revealed four deaths, three patients alive without disease, and one patient recently posttreatment with residual disease. The significance of the presence of the human papillomavirus in these individuals remains to be determined. The incidence of squamous cell carcinoma of the head and neck under age 45 is low, and whether there may be a higher incidence in HIV-positive patients cannot be determined from this small series.

Vestibular schwannomas (acoustic neuromas) are benign, slow-growing tumors of the eight cranial nerve. For patients who undergo surgical management in whom hearing preservation is not an issue, a translabyrinthine approach to these tumors is used. However, when hearing preservation is attempted, the two widely used approaches are the retrosigmoid (RS) approach and the middle fossa (MF) approach. Prior to 1996, our institution used the RS approach for hearing preservation attempts. In an effort to improve our hearing preservation rate, in 1996 we changed to the middle fossa (MF) approach for largely intracanalicular tumors with good hearing.