Primary melanoma of the petrous temporal bone.

Melanoma is a malignant tumor of melanocytes that is predominantly found in the skin. In rare cases, it arises from mucosal melanocytes. We describe a case of a solitary melanoma of the petrous apex of the temporal bone in a 67-year-old woman who presented with sudden hearing loss, aural fullness, and headaches, all on the right side. Magnetic resonance imaging identified a mass located at the right petrous apex; the lesion was hyperintense on T1-weighted imaging and isointense on T2 weighting, and it enhanced brightly with gadolinium contrast. The patient underwent removal of the lesion via a transcochlear approach with facial nerve translocation. Intra- and postoperative pathology identified a poorly differentiated malignancy consistent with a melanoma. Further investigations found no evidence of metastasis. Given a concern for residual disease, the patient was treated with radiation to the primary site. To the best of our knowledge, only 1 other case of primary melanoma of the petrous apex has been described in the literature.

Hearing outcomes in stapes surgery: a comparison of fat, fascia, and vein tissue seals.

OBJECTIVE: To compare short- and long-term hearing results following stapedectomy using 3 different oval window grafting materials with the same stapes prosthesis. STUDY DESIGN: Database review. SETTING: Tertiary referral private practice. SUBJECTS AND METHODS: Subjects were ears that underwent stapedectomy for otosclerosis, with placement of fat, fascia, or vein as an oval window seal and reconstruction with a titanium bucket handle prosthesis. A total of 365 procedures met these inclusion criteria: 98 fat grafts, 135 fascia grafts, and 132 vein grafts. Outcome measures included short-term (<1 year) and long-term follow-up air-bone gap. We compared the preoperative and postoperative amount of change in air-bone gap and preoperative and postoperative amount of change in the high-frequency bone conduction average. RESULTS: Overall median times to short-term and long-term follow-ups were 2.2 months and 36.1 months, respectively. There were no statistically significant differences between the 3 tissue seal groups in the amount of change in air-bone gap. There was no significant difference in amount of change in high-frequency bone conduction (representing sensorineural hearing level) between the 3 tissue seal groups. Most patients in all 3 groups had an air-bone gap at long-term follow-up of ≤ 10 dB (fat, 79.5%; fascia, 78.8%; and vein, 75.6%), with 90.3% of all patients at ≤ 20 dB. CONCLUSIONS: In both the short-term postoperative period and long-term follow-up, there were no significant differences in hearing results among 3 types of tissue seals of the oval window in stapes surgery. Fat, fascia, and vein grafts all provide satisfactory hearing outcomes in stapedectomy.

Porencephalic cyst: a review of the literature and management of a rare cause of cerebrospinal fluid otorrhea.

OBJECTIVE: To discuss the first reported case of spontaneous cerebrospinal fluid (CSF) otorrhea caused by a massive CSF-containing porencephalic cyst. STUDY DESIGN: A case report and review of the literature (MEDLINE 1962-2005). SETTING: A tertiary neurotologic referral center. PATIENT: A 65-year-old woman with congenital hemiplegia presented with left-sided spontaneous CSF otorrhea of 4-month duration. An audiogram revealed a mixed hearing loss. High-resolution computed tomography revealed a thinning of the tegmen and epitympanum without an obvious defect. Magnetic resonance imaging revealed a massive porencephalic cyst essentially replacing the entire left cerebral hemisphere. INTERVENTION: A transmastoid approach with three-layered closure was used to successfully repair the sieve-like defects that were discovered in her tegmen. RESULTS: The patient remains free of drainage, and the conductive hearing loss has resolved. CONCLUSION: Spontaneous CSF otorrhea caused by a porencephalic cyst is an uncommon cause of conductive hearing loss that has never been reported before. Only a few cases of traumatic CSF otorrhea/rhinorrhea associated with a porencephalic cyst have been reported. A high level of suspicion, a beta2-transferrin assay, and appropriate radiographic imaging are required for diagnosis in adults without a history of trauma, meningitis, chronic ear disease, or previous ear surgery.

Revision stapedectomy.

Revision stapedectomy can be a technically demanding operation. The surgeon must be prepared for many pathologic conditions before revision stapes surgery. Appropriate preoperative patient counseling is a must. The best chance for hearing improvement is in those cases that have a delayed conductive hearing loss after primary stapedectomy. This article serves as a guideline for discussing the myriad possibilities that may be encountered during this type of revision stapedectomy. Indications for revision stapedectomy and general surgical guidelines for management of specific pathologic conditions are discussed. The material presented is based on literature review, the authors' personal experience, and a review of temporal bone studies relative to stapes surgery.

Acoustic neuroma (vestibular schwannoma) revision.

The authors present their experience of more than 25 years, now in excess of 1200 patients, with cerebellopontine angle tumors. This article focuses on the management of planned subtotal resection of acoustic tumors in five subjects, and unexpected "residual" discovered by MRI scanning in 10 cases, which represents, to the best of the authors' knowledge, a residual rate of 1% of operated patients. The rate of residual tumor is as high as 19% in some series and, in part, depends on the surgical approach. For the purpose of this article, the authors did not include their cases of neurofibromatosis, because these tumors behave differently than unilateral sporadic schwannomas.

Should ossicular reconstruction be staged following tympanomastoidectomy.

OBJECTIVES: To determine whether ossicular reconstruction (OCR) performed concurrent with tympanomastoidectomy for cholesteatoma results in significantly different hearing results when compared to OCR performed in a separate, staged procedure. STUDY DESIGN: Retrospective. MATERIALS AND METHODS: Study subjects were patients undergoing OCR within a 2-year period. Intervention was OCR concurrent with tympanomastoidectomy for cholesteatoma (group 1) or staged following tympanomastoidectomy for cholesteatoma (group 2). Main outcome measures were mean postoperative air-bone gap (ABG), proportion achieving ABG closure to <20 dB and <30 dB for group 1 and group 2 overall and when controlling for type of mastoid cavity created (open or closed) and the status of the stapes suprastructure (TORP vs. PORP OCR). RESULTS: Eighty-three patients were identified. Forty underwent OCR concurrent with tympanomastoidectomy and 43 underwent a staged OCR after tympanomastoidectomy. Overall, similar hearing results were seen in the two groups. When considering the status of the stapes and mastoid cavity, concurrent OCR resulted in improved mean postoperative ABG in a closed mastoid cavity with an intact stapes suprastructure (P = .024). Furthermore, a greater proportion of patients who had concurrent OCR within this group achieved ABG <20 dB and ABG <30 dB, although this difference did not reach statistical significance (P = .092 and P = .078, respectively). By contrast, staged OCR resulted in improved mean postoperative ABG and ABG <30 dB in open mastoid cavities with an absent stapes suprastructure (P = .040, and P = .019, respectively). Similarly, a greater proportion achieved ABG <20 dB, approaching statistical significance (P = .055). CONCLUSIONS: Staged OCR is advantageous in those with most severe disease, whereas those with least severe disease may benefit from a concurrent OCR.

Trends in the diagnosis and the management of Meniere's disease: results of a survey.

OBJECTIVE: To determine the practices of the American Neurotology Society (ANS) membership in the evaluation and treatment of the Meniere's patient. STUDY DESIGN: Prospective. INTERVENTION: Questionnaire. MAIN OUTCOME MEASURE: Respondents' response to questions pertaining to the diagnostic and therapeutic practices in the management of Meniere's disease. RESULTS: Three hundred members of ANS were mailed a 15-item questionnaire. Two hundred three responded, for a 67.7% response rate. For the diagnosis of Meniere's disease, 1 in 3 practitioners relied solely on history, physical exam, and audiometry, whereas 2 in 3 relied in part on adjunctive tests, such as electrocochleography (ECOG) and electronystagmography (ENG). Two in 3 practitioners pursued retrocochlear studies on initial evaluation, with the overwhelming majority using MRI. In treating Meniere's disease, conservative medical management was preferred. For medically recalcitrant Meniere's disease, endolymphatic sac surgery (ESS) was the most commonly employed initial intervention (50%), followed by transtympanic gentamicin (38%). Currently, <10% routinely recommend the Meniett device. Eighty-three percent include ESS as a therapeutic option for medically recalcitrant Meniere's disease. The vast majority continue to perform surgical labyrinthectomies and vestibular nerve sections for Meniere's disease. CONCLUSIONS: Meniere's disease continues to pose a difficult diagnostic and therapeutic problem, resulting in heterogeneous approaches to both evaluation and treatment. Despite the 1995 American Academy of Otolaryngology guidelines in the diagnosis of Meniere's disease, most clinicians rely in part on ENG or ECOG in diagnosing Meniere's disease. Furthermore, despite the passing of 20 years since the publications claiming a purely placebo effect, ESS is the most commonly employed initial surgical treatment for Meniere's disease.

Electrocochleography in patients with Meniere's disease.

OBJECTIVE: The objective of this study was to evaluate the role of electrocochleography (ECoG) in the diagnosis of Meniere's disease. STUDY DESIGN: This study is a retrospective case review. METHODS: Patients undergoing ECoG for Meniere's disease between 1995 and 2003 were identified and segregated as having definite , probable , or possible Meniere's disease according to the 1995 American Academy of Otolaryngology-Head and Neck Surgery guidelines for Meniere's disease classification. Those determined to have probable and possible Meniere's disease were then combined to form a less-than-definite group for statistical analysis. Electrocochleography summating potential (SP)/action potential (AP) ratios were determined, and ratios greater than 0.4 were considered abnormal. The 2 groups were then compared to assess for any correlation between ECoG with the 1995 American Academy of Otolaryngology-Head and Neck Surgery Meniere's disease classification. RESULTS: Sixty patients with definite Meniere's disease and 37 with less-than-definite Meniere's disease were identified. Overall, 59.8% had abnormally elevated SP/AP ratios. Of those with definite Meniere's disease, 66.7% had abnormally elevated SP/AP ratios, whereas of those with less-than-definite Meniere's disease, 52.7% had abnormal ECoGs ( P = .069). CONCLUSIONS: A significant difference in ECoG results was not seen between the definite and less-than-definite Meniere's disease groups. Furthermore, approximately 30% of those with definite Meniere's disease would not be classified as having Meniere's disease based on ECoG results. Because of its lack of sensitivity, ECoG should not play a decisive role in determining the presence or absence of Meniere's disease.

Modifying cochlear implant design: advantages of placing a return electrode in the modiolus.

HYPOTHESIS: A modiolar return electrode significantly increases the current flow across spiral ganglion cells into the modiolus, and may decrease the cochlear implant's power requirements. BACKGROUND: Ideal cochlear implants should maximize current flow into the modiolus to stimulate auditory neurons. Previous efforts to facilitate current flow through the modiolus included the fabrication and use of precurved electrodes designed to "hug" the modiolus and silastic positioners designed to place the electrodes closer to the modiolus. In contrast to earlier efforts, this study explores the effects of return electrode placement on current distributions in the modiolus. METHODS: The effects of return electrode positioning on current flow in the modiolus were studied in a Plexiglas model of the cochlea. Results of model measurements were confirmed by measurements in the modiolus of human temporal bones. The return electrode was placed either within the modiolus, or remotely, outside the temporal bone, simulating contemporary cochlear implant configurations using monopolar stimulation. RESULTS: Cochlear model results clearly show that modiolar current amplitudes can be influenced significantly by the location of the return electrode, being larger when placed into the modiolus. Temporal bone data show similar findings. Voltages recorded in the modiolus are, on average, 2.8 times higher with the return electrode in the modiolus compared with return electrode locations outside the temporal bone. CONCLUSION: Placing a cochlear implant's return electrode in the modiolus should significantly reduce its power consumption. Reducing power requirements should lead to improved efficiency, safer long-term use, and longer device life.

Long-term effects of cerebellar retraction in the microsurgical resection of vestibular schwannomas.

OBJECTIVE: To determine the long-term effects, if any, of the greater cerebellar retraction that is required for retrosigmoid vestibular schwannoma versus resection as compared with the minimal, if any, cerebellar retraction required for translabyrinthine versus resection. STUDY DESIGN: Retrospective case control. METHODS: All patients who underwent retrosigmoid versus resection between 1988 to 2000 by one surgeon were identified. These patients were contacted and asked to complete the Dizziness Handicap Inventory (DHI). These patients were then matched to patients who underwent translabyrinthine versus resection according to age at surgery, age at time of study, sex, and size of tumor. Total DHI and subcategory DHI scores were compared using the Student t test. RESULTS: Thirty-three patients underwent the retrosigmoid approach, and 27 were contacted for completion of the survey, with a 81.3% response rate. Forty-six patients who underwent translabyrinthine versus resection were matched to the retrosigmoid group. Thirty-six were contacted and completed the survey for a 75% response rate. Twenty-seven patients that most closely matched the retrosigmoid group comprised the translabyrinthine group used for comparison. Minimal differences were seen between the two groups. Mean total DHI score for the retrosigmoid group was 17.6, which was not significantly different from the mean score of 16.8 seen in the translabyrinthine group (P =.888). When comparing small tumors (<1.5 cm), total DHI scores were not found to be significantly different (P =.859). We observed similar findings in those with intermediate-sized tumors (1.5-3 cm). CONCLUSION: The significantly greater cerebellar retraction that is necessary for retrosigmoid versus resection does not result in greater long-term balance and disability as compared with those patients who have undergone the translabyrinthine approach.

Comparison of postoperative facial nerve outcomes between translabyrinthine and retrosigmoid approaches in matched-pair patients.

OBJECTIVES/HYPOTHESIS: The objective was to assess whether the translabyrinthine approach for acoustic tumor removal offers better postoperative facial nerve function compared with the retrosigmoid approach. STUDY DESIGN: Retrospective case review from a tertiary otology referral center. METHODS: Patients who had undergone either retrosigmoid or translabyrinthine approach for removal of acoustic neuroma from January 1, 1980, to December 31, 1999, were included in the study. Two groups of patients were created, one containing retrosigmoid cases and the other, translabyrinthine. Attempts were made to match each retrosigmoid case to a translabyrinthine case with regard to tumor size, patient age, and date of operation. This matching served to eliminate these variables from influencing postoperative facial nerve outcomes. From an initial pool of 450 patients, 35 pairs of patients were matched for the study. Facial nerve functions were reported at immediate, 3-month, and 1-year postoperative periods. RESULTS: Patient demographics demonstrated that matched patients had almost identical tumor size, patient age, and date of operation. Comparisons of postoperative facial nerve functions between the matched groups revealed that retrosigmoid approach carried 2.86 times higher risk of facial nerve dysfunction during the immediate postoperative period. However, by 1 year, the facial nerve outcomes were similar between the two groups. CONCLUSION: Compared with the translabyrinthine approach, retrosigmoid approach carries a higher risk of postoperative facial nerve dysfunction during the immediate postoperative period. However, long-term facial nerve outcomes are identical between the two approaches.

Comparison of facial nerve function results after translabyrinthine and retrosigmoid approach in medium-sized tumors.

OBJECTIVE: To compare postoperative facial nerve function results according to surgical approach. STUDY DESIGN: Retrospective case review study. SETTING: All surgical procedures were conducted in collaboration with a neurosurgery team in teaching hospitals with an academic affiliation. PATIENTS: Patients with medium to large vestibular schwannomas, with the tumor size ranging from 2 to 3 cm. Ninety-eight patients were identified from an "Acoustic Neuroma Database" (date range of search, 1983-2000). MAIN OUTCOME MEASURES: The House-Brackmann scale was used for grading facial function in the immediate postoperative period and 1 year after. Guidelines of the American Academy of Otolaryngology-Head and Neck Surgery were used for classification of hearing preservation. RESULTS: Of the 98 patients, 17 were operated on through a retrosigmoid approach and 81 through the translabyrinthine route. The mean +/- SD ages of these 2 groups of patients were 46 +/- 13 and 51 +/- 14 years, respectively; mean +/- SD tumor sizes were 2.5 +/- 0.27 and 2.6 +/- 0.28 cm, respectively. One year after tumor removal via retrosigmoid approach, 10 (59%) of the 17 patients had good (grade I-II) facial functions and 2 (12%) had poor (grade V-VI) function. In the translabyrinthine group, 54 (68%) of 79 patients (2 patients had subtotal total tumor removal) had good facial nerve function at the end of the 1-year follow-up, and 13 (17%) continued to have poor facial function. The difference between these groups was not statistically significant (P>.05). Hearing was preserved in 4 (24%) of the 17 patients in the retrosigmoid group. CONCLUSION: Although the translabyrinthine approach may offer better long-term facial function compared with the retrosigmoid approach in patients with medium-sized tumors, the difference between these 2 groups was not significant enough to favor one approach over the other.

Early results with titanium ossicular implants.

OBJECTIVE: To report the efficacy of titanium middle ear prosthesis for ossicular reconstruction. STUDY DESIGN: Retrospective chart reviews were performed for 25 patients who had undergone titanium ossicular implants between January 1, 1999, and June 1, 20001. SETTING: Tertiary otology referral center. PATIENTS: All patients had a minimum of 6 months of postoperative follow-up and no evidence of recurrent otologic disease. INTERVENTION: All patients had undergone ossiculoplasty using titanium middle ear implants. MAIN OUTCOME MEASURES: Comparisons of preoperative and postoperative pure tone averages were performed. Air-bone gap closures and implant extrusion rates were measured. RESULTS Overall mean pure tone averages improved 22.2 dB with air-bone gap improvement at 20.9 dB. Fifty-six percent of patients achieved air-bone gap less than 20 dB postoperatively. The overall extrusion rate was 4%. However, with the placement of cartilage graft interposed between the prosthesis and the tympanic membrane, no extrusion was observed. CONCLUSION: Titanium implants provide comparable hearing improvement compared with other materials. The extrusion rate seems quite low if cartilage interposition graft is inserted. Its ease of handling, biocompatible properties, and sound conducting properties improve its efficacy as an ossicular implant.

Long-term results of small fenestra stapedectomy compared with large fenestra technique.

OBJECTIVES/HYPOTHESIS: A comparison is made of the postoperative long-term hearing results of small fenestra stapedectomy versus large fenestra techniques. Several reports in the literature favor improved hearing results in small fenestra stapedectomy compared with large fenestration and/or total stapedectomy. METHODS: A retrospective review of 34 patients who had stapedectomy performed by the same surgeon between 1982 and 1992. All patients have been followed for up to 10 years postoperatively. RESULTS: In 18 ears of 17 patients, surgery was performed with small fenestra technique, and 17 ears of 17 patients had total stapedectomy or posterior-half fenestration. After small fenestra stapedectomy, initial average air-bone gap at 500, 1000, and 2000 Hz was 9 dB hearing loss. Hearing thresholds at 4000 Hz deteriorated to 4% over the study period. Speech reception thresholds declined 15%. After total or posterior-half stapedectomy the average air-bone gap at 500, 1000, and 2000 Hz was 5 dB hearing loss. Hearing thresholds at 4000 Hz deteriorated 8% and 18% for the small fenestra and large fenestra groups, respectively, over time. Statistically, there was no difference in hearing deterioration rate between these two groups. CONCLUSIONS: An experienced surgeon can obtain excellent results using either large or small fenestra technique for otosclerosis. After initial successful closure of the air-bone gaps at all measured frequencies, hearing thresholds over the study period were stable for both groups.

Auditory and facial nerve function following surgery for cerebellopontine angle meningiomas.

OBJECTIVE: To investigate the postoperative auditory and facial nerve function results after cerebellopontine angle meningioma removal. DESIGN: Retrospective chart review. SETTING: Tertiary care referral center. PATIENTS: Twenty-one patients undergoing surgical removal of cerebellopontine angle meningiomas by the senior author (R.J.W.). INTERVENTIONS: Translabyrinthine or retrosigmoid approach for tumor extirpation. MAIN OUTCOME MEASURES: Postoperative auditory (pure-tone average and speech discrimination score) and facial (House-Brackmann scale) function within 1 year of follow-up. RESULTS: Twenty-three operations were performed on 21 patients. Hearing preservation through the retrosigmoid approach was attempted in 11 patients (48%). Normal hearing (class A) was preserved in 9 of 10 patients. Normal postoperative facial nerve function (House-Brackmann grade I) was conserved in 11 (65%) of 17 patients. CONCLUSIONS: This review demonstrates that successful hearing preservation is possible with meningiomas. Therefore, the retrosigmoid approach should be used whenever serviceable hearing is present preoperatively. Normal facial nerve function can also be preserved in the majority of patients.

Translabyrinthine approach for the management of large and giant vestibular schwannomas.

BACKGROUND: The removal of large vestibular schwannomas through the translabyrinthine approach is still controversial. MATERIALS AND METHODS: A retrospective review was performed of 81 patients (58 men and 23 women) with vestibular schwannomas 3 cm or greater, who underwent tumor removal via the translabyrinthine approach between 1985 and 2000. The mean tumor size was 3.7 +/- 0.81 cm, and the mean age of the patients was 47 +/- 16.1 years. The largest tumor was 6 cm. All surgical procedures were performed in collaboration with a neurosurgery team. RESULTS: Total tumor removal was accomplished in 77 cases (95.1%). The facial nerve was preserved anatomically in 69 (85.2%) of the patients. In 4 patients, divided nerves were repaired by primary anastomosis. Facial nerve function was assessed immediately after surgery and 1 year or more after discharge. Good function (House-Brackmann facial nerve Grade I or II) was present in 45% of patients and acceptable function (Grades I-IV) in 80% of patients 1 year after resection of the tumor. Cerebrospinal fluid leakage occurred in 12 patients (17%), meningitis developed in 3 patients (4%), and 1 patient experienced a stroke immediately after surgery. There were no deaths caused by surgery in this series. CONCLUSION: The translabyrinthine approach offers an excellent anatomical view of the cerebellopontine angle and a direct approach to the tumor with functional preservation of the facial nerve. Total removal is accomplished in most cases, with minimum incidence of morbidity and no incidence of mortality.

Increased CD4+ T cells during acute attack of Ménière's disease.

OBJECTIVE: We aimed to investigate if there is an alteration in CD4 and CD8 T-cell populations and in CD25 and CD49d activation antigens linked to these cells during an acute attack of Ménière's disease (MD). MATERIAL AND METHODS: Patients with MD who met the American Academy of Otolaryngology-Head and Neck Surgery criteria for the disease and healthy controls were enrolled in this study. Blood samples were collected during an acute attack of MD and when the disease was in its quiescent phase. RESULTS: 16 patients with MD and nine healthy controls were enrolled. The percentage of CD4 (T helper) cells was significantly increased during an acute attack of MD compared to that in healthy controls and in patients in the quiescent phase (71% vs 58% and 59%, respectively; p < 0.05). The increase in activation antigens was not statistically significant. CONCLUSION: To our knowledge this is the first demonstration of an increase in CD4 cells during an acute attack of MD. Further investigation is required to clarify the role of T cells in the pathogenesis of the disease.