Non-Exenteration Management of Sino-Orbital Fungal Disease.

PURPOSE: To describe the non-exenteration management of sino-orbital fungal infection, a life-threatening condition for which orbital exenteration is generally considered a first-line treatment. METHODS: A retrospective case series is presented of 7 orbits in 6 consecutive patients admitted and treated at 2 major metropolitan tertiary teaching hospitals in Sydney, New South Wales, Australia. RESULTS: Seven orbits in 6 consecutive patients with sino-orbital fungal infection were treated conservatively with surgical debridement and intravenous antifungal agents. Four patients were immunosuppressed and the other 2 patients were otherwise healthy. All presented with pain, proptosis, or loss of vision. Causative organisms found were Mucormycoses, Aspergillus, and Scedosporium prolificans. Exenteration was avoided in all patients as part of their planned management and 5 patients, including 1 with bilateral disease, survived their disease without exenteration. Medical treatment included intravenous liposomal amphotericin B or voriconazole. A single immunosuppressed patient deteriorated and as a last resort, exenteration was performed, but this made no difference to his clinical course and in retrospect could have been avoided as he died of multiple cerebral metastases diagnosed shortly after his deterioration. CONCLUSION: The authors recommend that patients with sino-orbital fungal disease preferably be treated conservatively, without orbital exenteration.

Rationale for the Development of a Novel Clinical Grading Scale for Postoperative Facial Nerve Function: Results of a Multidisciplinary International Working Group.

OBJECTIVE: The objective of the current study was to present the results of an international working group survey identifying perceived limitations of existing facial nerve grading scales to inform the development of a novel grading scale for assessing early postoperative facial paralysis that incorporates regional scoring and is anchored in recovery prognosis and risk of associated complications. STUDY DESIGN: Survey. SETTING: A working group of 48 multidisciplinary clinicians with expertise in skull base, cerebellopontine angle, temporal bone, or parotid gland surgery. RESULTS: House-Brackmann grade is the most widely used system to assess facial nerve function among working group members (81%), although more than half (54%) agreed that the system they currently use does not adequately estimate the risk of associated complications, such as corneal injury, and confidence in interrater and intrarater reliability is generally low. Simplicity was ranked as the most important attribute of a novel postoperative facial nerve grading system to increase the likelihood of adoption, followed by reliability and accuracy. There was widespread consensus (91%) that the eye is the most critical facial region to focus on in the early postoperative setting. CONCLUSIONS: Members were invited to submit proposed grading systems in alignment with the objectives of the working group for subsequent validation. From these data, we plan to develop a simple, clinically anchored, and reproducible staging system with regional scoring for assessing early postoperative facial nerve function after surgery of the skull base, cerebellopontine angle, temporal bone, or parotid gland.

The human vestibulo-ocular reflex and compensatory saccades in schwannoma patients before and after vestibular nerve section.

OBJECTIVE: To examine the vestibulo-ocular reflex (VOR) and compensatory-saccades before and after complete unilateral vestibular deafferentation (UVD). METHODS: Forty patients were studied before and after surgery for vestibular or facial schwannoma using the video head-impulse test (vHIT) and multivariable regression. RESULTS: Prior to UVD (median(IQR), 14(58.4) days), the average VOR-gain towards the lesioned-ear was lower than in normal for all semicircular canals (lateral, anterior, posterior: 0.69, 0.72, 0.49). One-week after UVD (5(3.0) days) VOR gains were further reduced (0.22, 0.37, 0.27), however, within one-year after UVD (171(125.0) days) the lesioned-ear VOR gains had slightly increased (+0.08, +0.11, +0.03), maximally for the anterior-canal. After UVD, the VOR gain asymmetry (gain towards minus away from intact-ear) was lower for the intact posterior-canal plane (0.56, 0.56, 0.22). For the lesioned canals, the frequency and amplitude of the first compensatory-saccade increased from 61-93% and 1.9-3.6° pre-surgery, to 98-99% and to 3.1-5.9° one-week post-surgery and remained unchanged over one-year; second saccade frequency and amplitude decreased over the same timespan. CONCLUSIONS: After UVD the high-acceleration VOR for the intact posterior-canal plane is more symmetrical than the other canals. First compensatory-saccades adapt within one week and subsequently change only marginally. SIGNIFICANCE: Saccade compensation from surgical UVD is near complete by one-week.

Cochlear Implantation in Ménière's Disease With and Without Labyrinthectomy.

OBJECTIVE: To investigate outcomes of cochlear implantation (CI) in patients with Ménière's disease (MD) with and without surgical labyrinthectomy. STUDY DESIGN: Retrospective study. SETTING: Multiple tertiary referral centers. SUBJECTS: Thirty one ears from 27 patients (17 men, 10 women, aged 42-84) with CI in ipsilateral MD ear. INTERVENTION: CI in ears with intact labyrinths (Group 1), CI with simultaneous surgical labyrinthectomy (Group 2), and CI sequential to surgical labyrinthectomy (Group 3). MAIN OUTCOME MEASURE: Within-subject improvement on Bamford Kowal Bench test or City University of New York open set sentence tests. RESULTS: Majority of ears achieved excellent open-set speech recognition by 12 months post-CI, irrespective of intervention group. Preoperative details including patient age and sex, implant, MD and previous intervention, and audiological test results did not significantly affect outcomes. Patients with MD undergoing CI only may experience vestibular dysfunction which may cause long-term concerns. Incidental finding was noted of eight ears with fluctuating symptoms in ipsilateral ear during 12-month period post-CI, with five of eight ears showing objective fluctuating impedances and mapping. CONCLUSION: CI in MD can yield good hearing outcomes in all three groups and this is possible even after a long delay after labyrinthectomy. Bilateral MD patients are complex and prospective quality of life (QoL) measures would be beneficial in being better able to manage the vestibular outcomes as well as the audiological ones.

Conservative management of acoustic neuroma.

AIM OF STUDY: To identify those patients with vestibular schwannoma (acoustic neuroma) in whom treatment becomes necessary. METHOD: Retrospective chart review. RESULT: A total of 205 patients with small tumors were followed for a mean of 40.8 months. The longest follow-up was 180 months. One hundred and ninety-seven patients had a follow-up of more than 12 months. Eight patients with a follow-up of less than 12 months were excluded from the study. In 136 patients (66.3%) the tumor did not grow. Forty-seven patients (23.9%) showed some evidence of slow growth. Eight of 197 patients (4%) had rapid growth and 6 patients (3%) had radiological evidence of tumor regression. Fifteen patients came to surgery. Five of these showed rapid growth, four developed ataxia in whom tumor growth was slow, three had ataxia without tumor growth, two patients developed brainstem compression, and one patient elected to proceed to surgery, although there were no tumor growth or symptoms. CONCLUSION: Few patients with small tumors will come to surgery in the short term. Perhaps the majority of patients with such small tumors will not need surgery. Long-term follow-up studies of 20 years or more are required to be come more confident about the natural history of these tumors. This study continues.

Spinal tumors in neurofibromatosis type 2. Is emerging knowledge of genotype predictive of natural history?

OBJECT: The authors conducted a study to examine the incidence, classification, and progression of spinal tumors in patients with neurofibromatosis Type 2 (NF2) treated at a single center, and to examine relationships with the known mutational subtypes of NF2. METHODS: They performed a retrospective review of clinical records, neuroimaging studies, and genetic data obtained in 61 patients with NF2. Forty-one (67%) of 61 patients harbored one or more spinal tumors. Thirty-four patients had undergone serial spinal magnetic resonance imaging during a mean follow-up period of 52 months (range 10-103 months; median 53 months). In 16 patients there were multiple extramedullary tumors smaller than 5 mm, which did not progress. Fourteen patients harbored at least one extramedullary tumor that was greater than 5 mm; of these, radiological progression was demonstrated or spinal tumor excision was performed during the follow-up period in eight cases (57%). Eleven patients harbored intramedullary cord tumors in addition to small and large extramedullary tumors, three (27%) of which exhibited radiological progression. In cases in which genotypes were known, protein-truncating mutations were significantly more likely to be associated with the presence of spinal tumors than in other types (p = 0.03, Fisher exact test). No associations between clinical behavior of spinal tumors and genotype, however, could be demonstrated. CONCLUSIONS: Spinal tumors in cases involving NF2 are heterogeneous in type, distribution, and behavior but larger-size tumors are more likely to progress significantly. Intramedullary tumors usually accompany multiple extramedullary tumors. In the authors' experience subtyping of the NF2 mutation has not yet influenced management. Protein-truncating mutations are associated with an increased prevalence of spinal tumors.

The Prevalence of Cochlear Obliteration After Labyrinthectomy Using Magnetic Resonance Imaging and the Implications for Cochlear Implantation.

OBJECTIVES: The aim of this study was to determine the prevalence of cochlear obliteration after labyrinthectomy. STUDY DESIGN: Retrospective review of medical records. SETTING: Tertiary referral center. PATIENTS: Sixty-five patients who had previously undergone resection of an acoustic neuroma through a translabyrinthine approach. INTERVENTION: The magnetic resonance studies were analyzed as part of the routine surveillance after tumor resection. MAIN OUTCOME MEASURE: To determine whether the cochlear lumen remained fluid filled. RESULTS: Sixty-five cochleas were examined. Forty-four cochleas were patent and, of the remaining 21, 9 were obliterated and 12 were partially obliterated. The average length of follow-up for the patent, partially obliterated, and obliterated cochlea groups was 47, 29, and 77 months, respectively. CONCLUSION: Obliteration of the cochlea, either partially or completely, occurs in approximately one-third of patients. Time elapsed after labyrinthectomy did not seem to be a predictor of cochlear obliteration.

Quality of Life in Vestibular Schwannoma Patients Managed by Surgical or Conservative Approaches.

OBJECTIVE: The aim of our study was to assess quality of life (QOL) among patients who underwent microsurgical excision of vestibular schwannoma (VS) compared with those managed conservatively. STUDY DESIGN: Retrospective study. SETTING: Tertiary care center. PATIENTS: There was a total sample population of 376 patients diagnosed with a unilateral VS. INTERVENTION: A total of 223 patients with unilateral VS returned the mailed questionnaires. These were then divided into two groups-78 that had undergone microsurgical excision and 145 that were managed conservatively. Subgroups within these primary groups were created for analysis. MAIN OUTCOME MEASURE: The primary outcome measure was the Medical Outcomes Study 36 Items Short Form (SF-36). The Dizziness Handicap Inventory test, Hearing Handicap Inventory test, and Tinnitus Handicap Inventory were also used. RESULTS: The surgically managed group had a worse QOL when compared with the conservatively managed group using SF-36, significantly so in the domains of physical role limitation and social functioning. Trends were seen toward a better QOL in some domains in the subgroups of male patients and patients younger than 65 years. Worse QOL scores in the Tinnitus Handicap Inventory were seen in the subgroups with larger tumor size. Finally, on correlation analysis between all handicap inventories and SF-36, handicap due to disequilibrium had the strongest correlation with worsening of QOL. In SF-36, the vitality domain showed the greatest correlation with otologic handicap overall, whereas the role emotional domain showed the least. CONCLUSION: This study found that worse QOL scores for surgically managed versus conservatively managed VS patients are most significant in the areas of physical role limitation and social functioning. In some areas, patients who are male and younger report better QOL. Handicap due to disequilibrium seems to have the greatest negative impact on QOL. These factors should be considered when counseling patients regarding approach to VS, in the context of an experienced management program.

Management of cystic facial neuromas: an alternative approach.

OBJECTIVE: To describe the treatment of cystic facial neuroma with drainage and marsupialization as an alternative to tumor removal and facial nerve grafting. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral hospital. PATIENTS: Two cases of cystic facial neuroma. MAIN OUTCOME MEASURES: Maintenance of facial nerve function at or better than preoperative function. RESULTS: Both cases were managed with cyst drainage. Case 1 retained House-Brackmann Grade I facial nerve function. Case 2 retained Grade II function. CONCLUSION: Cyst drainage provides a useful alternative to tumor removal and nerve sacrifice in patients with predominantly cystic facial neuromas and serviceable facial function.

The use of cone-beam computed tomography to determine cochlear implant electrode position in human temporal bones.

OBJECTIVE: To assess the utility of cone-beam computed tomography (CBCT) imaging in the estimation of cochlear implant (CI) electrode position in implanted temporal bones. STUDY DESIGN: Eight fresh frozen temporal bones were mounted and oriented as for standard surgery and were implanted with Cochlear Slim-Straight (SS) or Contour Advance electrode arrays by 2 CI surgeons. The bones were then imaged using an Accuitomo F170 CBCT scanner (isometric 250 µm voxel size) and were then processed for histologic sectioning (500 µm sections). MAIN OUTCOME MEASURES: The CBCT images and the histologic micrographs (providing the "gold standard") were examined independently by several observers who assessed the scalar position (tympani or vestibuli) of each electrode in each temporal bone specimen. RESULTS: Examination of the histologic micrographs confirmed that all electrodes were positioned within the scala tympani in all 8 bones. Similar judgments were made by the observers rating the CBCT images, except that one of the 2 observers estimated some of the apical electrodes to be located in the scala vestibuli in two of the bones implanted with the SS electrode. CONCLUSION: Cone-beam CT imaging is able to provide a good indication of the scalar position of implanted electrodes, although estimation may be slightly less reliable for apical electrodes and for straight electrode designs. Additional advantages of using CBCT for this purpose are shorter acquisition time and reduction of radiation dose as compared with conventional CT.

The use of cone beam imaging to determine cochlear implant electrode position in human temporal bones.

This paper describes the outcomes of cochlear implantation in eight cadaveric temporal bones using cone-beam CT imaging and histological examination with respect to scalar position of the electrode array.