Bevacizumab for Hearing Preservation in Neurofibromatosis Type 2: Emphasis on Patient-Reported Outcomes and Toxicities.

OBJECTIVE: Bevacizumab for hearing preservation in patients with neurofibromatosis type 2 (NF2) is an emerging practice. We set out to characterize the effectiveness and toxicity of bevacizumab in our patient group. STUDY DESIGN: Case series with chart review. SETTING: Tertiary referral center. SUBJECTS AND METHODS: Seventeen consecutive patients with NF2 received bevacizumab treatment for vestibular schwannomas, including 2 patients treated to maintain cochlear implant performance. Volumetric analysis of serial magnetic resonance imaging scans was used to evaluate radiographic response, and hearing response was evaluated with serial audiograms. Patient-reported outcomes were also assessed, including subjective hearing improvement, changes in tinnitus, vertigo, headaches, ear pain, and improvement in ability to communicate via telephone. RESULTS: A positive radiographic response occurred in 8 of 17 (47%) patients and the median tumor volume change was a tumor decrease of 19%. A positive hearing response was recorded in 5 of 9 (56%) patients. Two patients had a word recognition score improvement over 40%. There was an approximately 40% improvement in patient-reported outcomes. Primary toxicities included hypertension, proteinuria, dysgeusia, and amenorrhea. CONCLUSION: Bevacizumab treatment was followed by hearing improvement in 56% of patients, while decreased tumor volume was noted in 47%. These outcomes agree favorably with prior reported series. There were significant improvements in patient-reported outcomes that have not been described previously.

Randomized Controlled Pilot Study of Video Self-assessment for Resident Mastoidectomy Training.

A prospective randomized controlled pilot study was performed to determine if video self-assessment improves competency in mastoidectomy and to assess interrater agreement between expert and resident evaluations of recorded mastoidectomy. Sixteen otolaryngology residents were recorded while performing cadaveric mastoidectomy and randomized into video self-assessment and control groups. All residents performed a second recorded mastoidectomy. Performance was evaluated by blinded experts with a validated assessment scale. Video self-assessment did not lead to greater skill improvement between the first and second mastoidectomy. Interrater agreement was fair to substantial between the expert evaluators and between resident self-evaluations by recall and video review. Agreement between experts and residents was only slight to fair; residents consistently rated their performance higher than experts (P < .05). In conclusion, 1 session of video self-review did not lead to improved competence in mastoidectomy over standard practice. While experts agree on assessments, residents may overestimate their competency in performing cadaveric mastoidectomy.

Quantitative Proteomics of Vestibular Schwannoma Cerebrospinal Fluid: A Pilot Study.

This pilot study aimed to identify candidate proteins for future study that are differentially expressed in vestibular schwannoma (VS) cerebrospinal fluid (CSF) and to compare such proteins with those previously identified in perilymph and specimen secretions. CSF was collected intraoperatively prior to removal of untreated sporadic VS (3 translabyrinthine, 3 middle cranial fossa approaches) and compared with reference CSF samples. After proteolytic digestion and iTRAQ labeling, tandem mass spectrometry with ProteinPilot was used to identify candidate proteins. Of the 237 proteins detected, 13 were dysregulated in ≥3 of the 6 VS patients versus controls, and 13 were dysregulated (12 up, 1 down) in samples from patients with class D versus class B hearing. Four perilymph proteins of interest were dysregulated in ≥1 VS CSF samples. Thus, 26 candidate VS CSF biomarkers were identified that should be considered in future VS biomarker and tumor pathophysiology investigations.

Complication from hearing aid mold material: a case report and review of legal matters.

Ear mold impression middle ear foreign bodies are a rare complication of hearing aid fitting. Only a small number of cases have been reported; however, the actual incidence is unknown and likely much higher than expected. We present the case of a 77-year-old man with a history of preexisting tympanic membrane perforations who presented with an ear mold impression middle ear foreign body that required surgical removal. We explore the state and federal laws that guide the interaction between patients, hearing aid sellers and otolaryngologists. We highlight steps that can be taken to reduce the incidence of this complication.

Symptom timeline preceding cochlear implant failure: an institutional experience.

OBJECTIVE: Review cochlear explants and determine the incidence of device and medical failures and develop a pattern of symptoms indicating probable implant failure. STUDY DESIGN: Case series with chart review. SETTING: Tertiary referral center. SUBJECTS AND METHODS: Subjects were selected if they underwent cochlear explantation. Data were reviewed to determine initial symptom and symptom timeline preceding implant failure. RESULTS: A total of 847 implants were performed from 1988 to 2008, with 128 devices explanted. Of total implants, 72% were Advanced Bionic devices, and 28% were Cochlear Nucleus devices. Overall failure rate was 128 of 847 (15%), with 51 (6%) medical failures and 77 (9%) device failures. Patients with Advanced Bionic devices underwent 102 explants (16.7% failure rate), with 35% medical failures and 65% device failures. Patients with Nucleus devices underwent 26 explants (11% failure rate), with 58% medical failures and 42% device failures. Medical failures included infected devices and wound dehiscence, with the pattern averaging 4.5 months to explantation. Hard device failures included sudden malfunction and slow decline in function, with pattern of failure over 4.2 months. Soft failures included tinnitus and discomfort, with failure over 8 months. CONCLUSIONS: There were common patterns when evaluating both medical and device failures. By recognizing patterns of symptoms that may indicate probable implant failure, otolaryngologists can better counsel patients on what to expect with their implants when they present with certain symptoms.

Interleukin-8 production in response to tumor necrosis factor-alpha by cholesteatoma keratinocytes in cell culture.

OBJECTIVES/HYPOTHESIS: Keratinocytes harvested from acquired cholesteatoma and grown in cell culture will demonstrate increased interleukin-8 (IL-8) production in response to tumor necrosis factor (TNF)-alpha as compared with a control keratinocyte cell line. Immunohistochemical studies have identified IL-8 and TNF-alpha, mediators of bony destruction, in tissue samples of cholesteatoma. TNF-alpha stimulates IL-8 production in healthy epidermal keratinocyte cell lines. It is not known whether TNF-alpha stimulates IL-8 production in cultured cholesteatoma keratinocytes. STUDY DESIGN: Prospective controlled tissue culture experiment. METHODS: Tissue from an acquired cholesteatoma was dissociated and grown in keratinocyte serum-free media for 8 weeks. Cholesteatoma keratinocytes and a control cell line of skin epidermal keratinocytes were treated with TNF-alpha. Conditioned media were harvested; production of IL-8 was measured by enzyme-linked immunosorbent assay, and cell counts were performed. RESULTS: At a zero concentration of TNF-alpha, mean production of IL-8 by cholesteatoma keratinocytes was 39,809 pg/mL/24hr/1 × 10(6) cells versus 1,907 pg/mL/24hr/1 × 10(6) cells from skin epidermal keratinocytes, a statistically significant difference (P < .05). The cholesteatoma keratinocytes showed a 2.1-fold increase in response to 2 pg/mL of TNF-alpha and a 2.44-fold increase in response to 20 pg/mL of TNF-alpha. The skin epidermal keratinocyte cell line demonstrated a 1.07- and 1.13-fold increase to respective concentrations of TNF-alpha. CONCLUSIONS: Cholesteatoma keratinocytes appear to retain cell signaling characteristics in vitro that distinguish them from skin epidermal keratinocytes. This finding may indicate that cholesteatoma keratinocytes undergo a change in behavior in vivo that is preserved after the cells are removed from the inflammatory environment of the middle ear.

The first new otologic disorder in a century: superior canal dehiscence syndrome.

Superior canal dehiscence syndrome is a recently described vestibular and hearing condition caused by an absence of bone over the arc of the superior semicircular canal. Patients with the condition present with a variety of perplexing symptoms including conductive hearing loss, hyperacusis, vertigo, autophony, and eye movement in response to sound. This article presents the case of a patient with the condition, discusses how it is distinct from other vestibular and hearing disorders, and describes how patients with this disorder can be diagnosed and treated.

Late failure rate of hearing preservation after middle fossa approach for resection of vestibular schwannoma.

OBJECTIVE: To explore the long-term hearing results after a middle fossa approach for resection of vestibular schwannoma. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENTS: All patients undergoing a middle fossa approach for resection of vestibular schwannoma at a single institution with intent to preserve hearing between December 1989 and December 2009 were included in the study population. INTERVENTIONS: Standard middle fossa approach for resection of vestibular schwannoma, magnetic resonance imaging (MRI), and audiogram. MAIN OUTCOME MEASURES: Recurrence of tumor as evaluated by MRI and hearing results as measured by serial audiograms. RESULTS: Seventy-eight patients were identified who met study criteria. Fifty-one (65%) of the 78 patients had usable hearing (American Academy of Otolaryngology-Head and Neck Surgery [AAO-HNS] class A or B) postoperatively. Forty-four patients with successful hearing preservation had follow-up beyond their initial postoperative visit. Five (11%) of the 44 patients showed late degradation to nonserviceable hearing (AAO-HNS class C or D) over a mean follow-up of 4.0 years. Kaplan-Meier estimate of preservation of class A or B hearing at 10 years was 72%. Of the five patients with late degradation in hearing, 2 were found to have recurrences of their original tumor on MRI. There were 5 confirmed recurrences in the total study population. CONCLUSION: Late degradation of hearing was an infrequent occurrence after initially successful hearing preservation. When hearing degradation did occur, there seemed to be a correlation with tumor recurrence.

Implantable hearing devices.

PURPOSE OF REVIEW: Implantable hearing devices have been developed to address the shortcomings of traditional hearing aids. Currently, there are two US Food and Drug Administration clinical trials evaluating fully implantable hearing devices. RECENT FINDINGS: Implantable hearing devices attempt to improve upon stigma, cosmesis, occlusion effect, and feedback, as well as other factors that prevent patients from using traditional hearing aids. However, there are many challenges inherent in designing an implantable hearing device. Different devices have attempted to overcome these challenges in different ways. We will discuss these differences in design, their individual pros and cons as well as directions of future research. SUMMARY: Although implantable hearing devices are not currently available they do hold promise for patients not adequately served by conventional hearing aids.

Secreting jugulotympanic paraganglioma with venous involvement into the thorax.

Paragangliomas are highly vascular tumors that arise from chief cells in extra-adrenal paraganglia of the autonomic nervous system. Jugulotympanic paragangliomas involve the middle ear and jugular fossa. Secreting jugulotympanic paragangliomas with thoracic extension are rare. We report a case with unexpected involvement of the internal jugular vein. Otolaryngologists should be aware that the jugulotympanic paragangliomas traverse variable anatomic pathways. Multiple imaging options, including magnetic resonance venography, are useful and should include the neck for definitive characterization of tumor anatomy. Patients with refractory hypertension and masses suspicious for paraganglioma should be examined for functional tumors.

Infectious complications in pediatric cochlear implants.

OBJECTIVES: Infectious complications may cause significant delay in cochlear implant device initiation and programming and be a source of additional morbidity. We reviewed our experience with infectious complications in the pediatric age group to determine specific sources that may not be seen in adults. STUDY DESIGN: A retrospective analysis from a single implant center. METHODS: Cases of pediatric cochlear implants were reviewed for data on infectious complications. Complications were identified as "major" or "minor," "early" or "delayed." Information was gathered regarding any comorbid, chronic health condition. Data related to the causative organism(s) were collected. RESULTS: Two hundred sixty-eight cases of pediatric implants were reviewed. Twenty-two cases were identified (an infection rate of 8.2%), all classified as "major." The majority, 12, were classified as "delayed" complications. Twenty-one cases required explantation with 14 successfully reimplanted. Five cases (in 4 patients) or 23% were associated with a specific chronic pediatric condition including two children with tracheostomies. Among implanted children who had chronic health conditions, 42% developed implant-related infections. Among otherwise healthy implanted children, only 6.6% developed implant-related infections. Resistant bacterial infections were not identified. CONCLUSIONS: Health conditions in the pediatric age group were associated with 23% of our complications, a risk factor not previously identified in the literature. These children, demonstrating seven times the infection rate of healthy children, should be carefully observed postoperatively. Overall, cochlear implantation in children continues to be associated with a low risk of infectious complications.

Peripheral facial nerve regeneration using collagen conduit entubulation in a cat model.

OBJECTIVES: Facial nerve (FN) injuries are functionally, psychologically, and financially debilitating. Facial nerve autograft repairs produce significant donor nerve morbidity and functional results that rarely exceed House-Brackmann (HB) grade III over VI. In this study we sought to enhance FN regeneration via collagen conduit entubulation. METHODS: Five control cats underwent right ("cut-side") FN transection and immediate microsurgical anastomosis repair. Five experimental cats underwent identical repairs plus collagen conduit entubulation of each anastomosis. RESULTS: Postoperative behavioral observations revealed gradual FN functional recovery in all cats, who attained adapted HB grades of II to III over VI after 6 weeks. Electromyographic latencies and amplitudes from the bilateral orbicularis oculi and orbicularis oris muscles indicated restoration of FN continuity in all 10 cats. In comparison with FN repairs without conduits, repairs with conduits significantly enhanced recovery of amplitude in cut-side orbicularis oculi muscles (p = .037) and latency in cut-side orbicularis oris muscles (p = .048). In comparison with intact left ("uncut-side") FN latencies and amplitudes, more statistically significant differences in cut-side FN function were observed in repairs without conduits than in repairs with conduits. Conduits therefore facilitated a more complete return of electrophysiological function. Histologic analyses confirmed FN continuity and revealed more organized FN regenerative architecture in conduit-implanted repairs. CONCLUSIONS: The overall results support enhanced FN regeneration with collagen conduit entubulation.

Sarcoidosis presenting with hearing loss and granulomatous interstitial nephritis in an adolescent.

Granulomatous interstitial nephritis is an uncommon finding in a kidney biopsy. The differential diagnosis is broad and includes infections, drug exposure, and sarcoidosis. Sarcoidosis, a systemic disorder of unknown etiology characterized by the presence of noncaseating granulomata in affected organs, is rare in children. We discuss an adolescent boy with the unusual presentation of granulomatous interstitial nephritis and acute deafness. Sarcoidosis should be considered as part of the differential diagnosis for children and adolescents with hearing loss and kidney disease.

Predictors for resident success in otolaryngology.

BACKGROUND: This study aimed to determine predictors for otolaryngology resident success using data available at the time candidates are interviewed (eg, medical school attended, letters of recommendation, test scores) and data that emerge during residency. STUDY DESIGN: We performed a retrospective cohort study of 36 residents who entered our program between 1983 and 1993. RESULTS: Seventy percent of Alpha Omega Alpha (AOA) members and 13% of nonmembers were in the highest tertile based on faculty ranking (p<0.01), and candidates with an exceptional trait were more likely than those without an exceptional trait to rank in the highest tertile (57% versus 10%, p<0.01). AOA membership was also related to current academic appointment (p=0.02). Significant correlations included United States Medical Licensing Examination (USMLE) I score, year 2 in-training score (0.48, p=0.03), and years 3 and 4 in-training score and faculty ranking (minus 0.39, minus 0.50, respectively, p50% of the interviewers (p<0.05 for both). CONCLUSIONS: In our program designed to train academic otolaryngologists, postresident success was strongly predicted by having an exceptional trait and AOA membership. Success during residency was predicted by interviewer's impression of the candidate and a USMLE I score>570. Knowledge of these factors at the time of the resident interview could increase the likelihood of selecting the most appropriate candidates for academic otolaryngology. Resident success is a complex outcome, and other unmeasured and unexamined characteristics can provide additional insight into choosing successful residents.

Cochlear implantation in severe traumatic brain-injury and paranoid schizophrenia: two case studies.

In view of the demanding instruction and training requirements associated with cochlear implantation;,patients with cognitive impairments have typically been considered to be poor candidates for this procedure. This presumption persists in part because experience and research in this area are limited. We describe our experience with implanting cochlear devices in 2 patients who had significant psychological impairments; 1 patient had experienced a severe brain injury, and the other had paranoid schizophrenia. Nevertheless, both patients were able to follow the implant training program, and they experienced different degrees of improvement in their hearing. We discuss our preoperative evaluations of these patients and the key factors that led to our decision to proceed with surgery.

Chronic and recurrent otitis media: a genome scan for susceptibility loci.

Otitis media (OM) is the most common childhood disease. Almost all children experience at least one episode, but morbidity is greatest in children who experience chronic/recurrent OM (COME/ROM). There is mounting evidence that COME/ROM clusters in families and exhibits substantial heritability. Subjects who had tympanostomy tube surgery for COME/ROM (probands) and their families were recruited for the present study, and an ear examination was performed, without knowledge of the subject's history, to determine presence of OM sequelae. In addition, tympanometric testing was performed at three frequencies (226, 630 or 710, and 1,400 Hz) to detect abnormal middle-ear mechanics, and hearing was screened at 20 dB for the speech frequencies. Of these families, 121 had at least two individuals who had received the diagnosis of COME/ROM (364 affected and genotyped individuals), of whom 238 affected and informative relative pairs were used for analyses. Single-point nonparametric linkage analysis provided evidence of linkage of COME/ROM to chromosome 10q at marker D10S212 (LOD 3.78; P=3.0 x 10(-5)) and to chromosome 19q at marker D19S254 (LOD 2.61; P=5.3 x 10(-4)). Analyses conditional on support for linkage at chromosomes 10q and 19q resulted in a significant increase in LOD score support on chromosome 3p (between markers D3S4545 and D3S1259). These results suggest that risk of COME/ROM is determined by interactions between genes that reside in several candidate regions of the genome and are probably modulated by other environmental risk factors.

Complications of otitis media before placement of tympanostomy tubes in children.

OBJECTIVES: To report the incidence of short-term complications from otitis media in children before placement of tympanostomy tubes (TTs) and to compare children treated according to the Agency for Health Care Policy and Research guidelines with those who were treated earlier or later than recommended. DESIGN: Retrospective outcomes review. PATIENTS: Subjects were children aged 10 or younger who had TTs inserted at a tertiary care county hospital from January 1, 1999, to December 31, 2000. Exclusion criteria included prior TT placement, any concurrent head and neck procedure, and craniofacial defects. INTERVENTION: Tympanostomy tube placement. MAIN OUTCOME MEASURES: Any occurrences of otorrhea, tympanic membrane perforation, tinnitus, antibiotic reactions, speech or language delay, febrile seizures, or meningitis before placement of TTs documented in the county hospital records were recorded as complications. Hearing loss was considered separately. RESULTS: Of 147 children who met our criteria, 81 (55.1%) had 1 or more complications from otitis media before placement of TTs. Fifty-five (37.4%) had 2 to 6 complications documented. Adverse reactions to antibiotics were the most common complication, reported in 34 (23.1%). CONCLUSIONS: Most children in this county hospital experienced short-term complications of otitis media before receiving TTs. Even the children treated "on time" according to the guidelines from the Agency for Health Care Policy and Research experienced complications; however, adherence to the guidelines had no significant effect on complications.

Resolution of facial stimulation in otosclerotic cochlear implants.

OBJECTIVES: We examined the incidence of facial nerve stimulation in a population of patients with otosclerosis implanted with the Nucleus 22, Clarion 1.2, or Clarion High Focus (CHF) device. STUDY DESIGN AND SETTING: A retrospective chart review was used for 147 patients who had been implanted with electronic cochlear devices at the University of Minnesota between June 1986 and February 2001. Seventy-four patients were implanted with the Nucleus 22, 41 patients were implanted with the Clarion 1.2 (without a positioner), and 32 patients were implanted with the CHF I or II. RESULTS: Eleven (14.9%) of the 74 patients implanted with the Nucleus 22 device had facial stimulation. There were 9 patients with otosclerosis. Seven (78%) of the 9 patients with otosclerosis had facial stimulation. One (2.4%) of the 41 patients implanted with the Clarion 1.2 device (without a positioner) had facial stimulation and that 1 (100%) patient in the group had otosclerosis. There were 4 patients in the CHF group with otosclerosis. One patient experienced stimulation, but it was possible to decrease maximum current levels and still use the electrode pair. CONCLUSION: Facial stimulation appears to be less problematic in patients with otosclerosis implanted with the CHF devices.

Chordomas of the skull base: manifestations and management.

PURPOSE OF REVIEW: Chordomas are rare congenital tumors of the midline skull base. They are slow-growing and usually cause symptoms only when they compress a cranial nerve. RECENT FINDINGS: The MRI will show the lesion with irregular uptake of gadolinium. The only way to define the diagnosis is with tissue, and there are two primary pathologic forms of the disease. Resection approaches vary with extent of disease, surgeon preference, and critical structures involved. SUMMARY: There is building evidence that proton beam therapy improves survival and duration of a disease-free state.