Colin L. W. Driscoll

Patient Outcomes After Vestibular Schwannoma Management: a Prospective Comparison of Microsurgical Resection and Stereotactic Radiosurgery

OBJECTIVE The best management for patients with small- to medium-sized vestibular schwannomas (VS) is controversial. METHODS : A prospective cohort study of 82 patients with unilateral, unoperated VS less than 3 cm undergoing surgical resection (n = 36) or radiosurgery (n = 46). Patients undergoing resection were younger (48.2 yr versus 53.9 yr, P = 0.03). The groups were similar with regard to hearing loss, associated symptoms, and tumor size. The mean follow-up period was 42 months (range, 12-62 mo). RESULTS Normal facial movement and preservation of serviceable hearing was more frequent in the radiosurgical group at 3 months (P < 0.001), 1 year (P < 0.001), and at the last follow-up examination (P < 0.01) compared with the surgical resection group. Patients undergoing surgical resection had a significant decline in the following subscales of the Health Status Questionnaire 3 months after surgery: physical functioning (P = 0.006), role-physical (P < 0.001), energy/fatigue (P = 0.02), and overall physical component (P = 0.004). Patients in the surgical resection group continued to have a significant decline in the physical functioning (P = 0.04) and bodily pain (P = 0.04) subscales at 1 year and in bodily pain (P = 0.02) at the last follow-up examination. The radiosurgical group had no decline on any component of the Health Status Questionnaire after the procedure. The radiosurgical group had lower mean Dizziness Handicap Inventory scores (16.5 versus 8.4, P = 0.02) at the last follow-up examination. There was no difference in tumor control (100 versus 96%, P = 0.50). CONCLUSION Early outcomes were better for VS patients undergoing stereotactic radiosurgery compared with surgical resection (Level 2 evidence). Unless long-term follow-up evaluation shows frequent tumor progression at currently used radiation doses, radiosurgery should be considered the best management strategy for the majority of VS patients.

Cochlear Implants in Five Cases of Auditory Neuropathy: Postoperative Findings and Progress†

Objectives To review our experiences with some of the preoperative and postoperative findings in five children who were diagnosed with auditory neuropathy and were provided with cochlear implants. We describe changes in auditory function, which enabled these children to have significant improvement in their hearing and communication skills.

Costimulating aberrant T cell responses by B7-H1 autoantibodies in rheumatoid arthritis

A pathogenic hallmark of rheumatoid arthritis (RA) is persistent activation of self-reactive CD4(+) T cells. The cause of this aberrant activity remains elusive. We report here detection of autoantibodies against B7-H1, a recently described member of the B7 family, in 29% of patients with RA versus 4% of healthy donors. High-level expression of cell surface B7-H1 are found on activated human CD4(+), CD8(+), and CD45RO(+) T cells. Immobilized autoantibodies to B7-H1 are capable of costimulating the proliferation of CD4(+) T cells in vitro, and the presence of these autoantibodies correlates with active disease status. Using immobilized B7-H1 mAbs and programmed death 1Ig, we demonstrate that engagement of B7-H1 on CD4(+) T cells costimulates proliferation and secretion of IL-10, and subsequently leads to programmed cell death, accompanied with upregulated expression of TNF-related apoptosis-inducing ligand and activation of caspase-3. Taken together with our previous findings, these data indicate a bidirectional signaling role of B7-H1 in T cell costimulation and apoptosis and implicate B7-H1 autoantibodies as contributing to the progression of RA by inducing aberrant T cell responses.

Cogan Syndrome: A Retrospective Review of 60 Patients Throughout a Half Century

OBJECTIVE To evaluate the disease manifestations and clinical course of patients affected by Cogan syndrome (a syndrome of nonsyphilitic interstitial keratitis and vestibuloauditory symptoms) at a single institution during roughly a half century. PATIENTS AND METHODS Medical records of all patients diagnosed as having Cogan syndrome at the Mayo Clinic in Rochester, Minn, and who were followed up from 1940 to 2002 were comprehensively reviewed. Otolaryngologic, ophthalmologic, and systemic manifestations of disease were analyzed. Analysis included patient demographics, presenting manifestations, delayed manifestations, laboratory testing, physical examination features, therapeutic interventions, disease course, and hearing and vision outcomes. RESULTS Sixty patients were identified as having Cogan syndrome, with follow-up from 1940 to 2002. Most patients presented initially with vestibuloauditory symptoms, most commonly sudden hearing loss (50%). The most common inflammatory ophthalmologic condition noted was bilateral interstitial keratitis. Headache (40%), fever (27%), and arthralgia (35%) were the most frequently encountered systemic manifestations. Evidence of aortitis was found in 12% of patients. Complete hearing loss was eventually noted in 52% of affected patients, whereas permanent loss of any degree of vision was uncommon. Cochlear implantation outcomes were uniformly good. Death directly or indirectly attributed to the effects of Cogan syndrome was noted in 4 patients. CONCLUSIONS The major disease-related morbidities were due to vestibuloauditory disease and only infrequently due to systemic manifestations such as vasculitis, with or without aortitis. Cochlear implantation has been of major benefit in modern hearing rehabilitation for this patient population. We advise caution before institution of protracted courses of high-dose corticosteroids and/or chemotherapy for patients without pronounced systemic disease or severe eye disease unmanageable by topical or periocular corticosteroids alone.

Clinical Practice Guideline: Bell’s Palsy

Objective Bell’s palsy, named after the Scottish anatomist, Sir Charles Bell, is the most common acute mono-neuropathy, or disorder affecting a single nerve, and is the most common diagnosis associated with facial nerve weakness/paralysis. Bell’s palsy is a rapid unilateral facial nerve paresis (weakness) or paralysis (complete loss of movement) of unknown cause. The condition leads to the partial or complete inability to voluntarily move facial muscles on the affected side of the face. Although typically self-limited, the facial paresis/paralysis that occurs in Bell’s palsy may cause significant temporary oral incompetence and an inability to close the eyelid, leading to potential eye injury. Additional long-term poor outcomes do occur and can be devastating to the patient. Treatments are generally designed to improve facial function and facilitate recovery. There are myriad treatment options for Bell’s palsy, and some controversy exists regarding the effectiveness of several of these options, and there are consequent variations in care. In addition, numerous diagnostic tests available are used in the evaluation of patients with Bell’s palsy. Many of these tests are of questionable benefit in Bell’s palsy. Furthermore, while patients with Bell’s palsy enter the health care system with facial paresis/paralysis as a primary complaint, not all patients with facial paresis/paralysis have Bell’s palsy. It is a concern that patients with alternative underlying etiologies may be misdiagnosed or have unnecessary delay in diagnosis. All of these quality concerns provide an important opportunity for improvement in the diagnosis and management of patients with Bell’s palsy. Purpose The primary purpose of this guideline is to improve the accuracy of diagnosis for Bell’s palsy, to improve the quality of care and outcomes for patients with Bell’s palsy, and to decrease harmful variations in the evaluation and management of Bell’s palsy. This guideline addresses these needs by encouraging accurate and efficient diagnosis and treatment and, when applicable, facilitating patient follow-up to address the management of long-term sequelae or evaluation of new or worsening symptoms not indicative of Bell’s palsy. The guideline is intended for all clinicians in any setting who are likely to diagnose and manage patients with Bell’s palsy. The target population is inclusive of both adults and children presenting with Bell’s palsy. Action Statements The development group made a strong recommendation that (a) clinicians should assess the patient using history and physical examination to exclude identifiable causes of facial paresis or paralysis in patients presenting with acute-onset unilateral facial paresis or paralysis, (b) clinicians should prescribe oral steroids within 72 hours of symptom onset for Bell’s palsy patients 16 years and older, (c) clinicians should not prescribe oral antiviral therapy alone for patients with new-onset Bell’s palsy, and (d) clinicians should implement eye protection for Bell’s palsy patients with impaired eye closure. The panel made recommendations that (a) clinicians should not obtain routine laboratory testing in patients with new-onset Bell’s palsy, (b) clinicians should not routinely perform diagnostic imaging for patients with new-onset Bell’s palsy, (c) clinicians should not perform electrodiagnostic testing in Bell’s palsy patients with incomplete facial paralysis, and (d) clinicians should reassess or refer to a facial nerve specialist those Bell’s palsy patients with (1) new or worsening neurologic findings at any point, (2) ocular symptoms developing at any point, or (3) incomplete facial recovery 3 months after initial symptom onset. The development group provided the following options: (a) clinicians may offer oral antiviral therapy in addition to oral steroids within 72 hours of symptom onset for patients with Bell’s palsy, and (b) clinicians may offer electrodiagnostic testing to Bell’s palsy patients with complete facial paralysis. The development group offered the following no recommendations: (a) no recommendation can be made regarding surgical decompression for patients with Bell’s palsy, (b) no recommendation can be made regarding the effect of acupuncture in patients with Bell’s palsy, and (c) no recommendation can be made regarding the effect of physical therapy in patients with Bell’s palsy.

Implications of minimizing trauma during conventional cochlear implantation.

Objective: To describe the relationship between implantation-associated trauma and postoperative speech perception scores among adult and pediatric patients undergoing cochlear implantation using conventional length electrodes and minimally traumatic surgical techniques. Study Design: Retrospective chart review (2002-2010). Setting: Tertiary academic referral center. Patients: All subjects with significant preoperative low-frequency hearing (≤70 dB HL at 250 Hz) who underwent cochlear implantation with a newer generation implant electrode (Nucleus Contour Advance, Advanced Bionics HR90K [1J and Helix], and Med El Sonata standard H array) were reviewed. Intervention(s): Preimplant and postimplant audiometric thresholds and speech recognition scores were recorded using the electronic medical record. Main Outcome Measure(s): Postimplantation pure tone threshold shifts were used as a surrogate measure for extent of intracochlear injury and correlated with postoperative speech perception scores. Results: Between 2002 and 2010, 703 cochlear implant (CI) operations were performed. Data from 126 implants were included in the analysis. The mean preoperative low-frequency pure-tone average was 55.4 dB HL. Hearing preservation was observed in 55% of patients. Patients with hearing preservation were found to have significantly higher postoperative speech perception performance in the CI-only condition than those who lost all residual hearing. Conclusion: Conservation of acoustic hearing after conventional length cochlear implantation is unpredictable but remains a realistic goal. The combination of improved technology and refined surgical technique may allow for conservation of some residual hearing in more than 50% of patients. Germane to the conventional length CI recipient with substantial hearing loss, minimizing trauma allows for improved speech perception in the electric condition. These findings support the use of minimally traumatic techniques in all CI recipients, even those destined for electric-only stimulation.

Long-term follow-up of transtympanic gentamicin for Ménière's syndrome.

Objective Recent studies have shown that transtympanic gentamicin for Méniéres syndrome is effective. Current treatment protocols vary. One concept has been to perform a chemical ablation; the other has been to perform a chemical alteration. Ablation requires multiple injections and is effective in controlling the vertigo, but it is associated with a significant incidence of hearing loss. Chemical alteration uses a minimal dose to reduce vestibular function without affecting cochlear function. Study Design Prospective. Setting Tertiary medical center. Patients Patients had classic unilateral Méniéres syndrome that was unresponsive to medical therapy. Intervention A single injection of gentamicin is given, and the patient is seen 1 month after injection. If indicated, the patient receives another injection and is reevaluated 1 month later. Main Outcome Measures Control of vertigo and maintenance of hearing using the American Academy of Otolaryngology–Head and Neck Surgery (AAO-HNS) guidelines (1995). Results Fifty-six patients have documented follow-up for 2 years or more, and 21 have 4 years or more of follow-up. This article presents the 4-year results as outlined by the AAO-HNS guidelines. Vertigo classes A and B were seen in 82% of patients. The patients followed 2 to 4 years had 86% vertigo class A and B results. Those followed 4 years or more show 76% with a vertigo class A or B result. In this study there has been minimal cochlear loss. There was vestibular change clinically, which was documented by electronystagmography. Conclusions It appears that a single transtympanic gentamicin injection is effective in controlling the vertigo of Méniéres syndrome. Cochlear impact has been minimal. It is most useful for those patients who have failed medical management and are severely affected but not totally incapacitated by the disease.

Significance of House-Brackmann facial nerve grading global score in the setting of differential facial nerve function

Objective To determine the clinical significance of the House-Brackmann facial nerve grading scale (HBFNGS) in the setting of differential function along the branches of the facial nerve. Study Design Prospective study of 38 patients with facial palsy who demonstrated differential facial function. Setting Tertiary referral center. Patients Patients with facial nerve dysfunction from any cause. Patients with complete facial nerve paralysis (House-Brackmann Grade 6) were excluded. Intervention Physicians were provided with printed description of the HBFNGS and asked to report facial nerve function as a traditional global score and as a regional score based on the House-Brackmann scale for the forehead, eye, nose, and mouth. This was reported as FwExNyMz, where w, x, y, and z ranged from 1 to 6 based on the HBFNGS. Synkinesis was graded as none, mild, or severe. Main Outcome Measures The traditional HBFNGS score was compared with a regional grading facial nerve grading system based on the HBFNGS for the forehead, eye, nose, and mouth. Agreement between the traditional global score and the regional scores was analyzed. Results In patients with variable facial weakness, the single House-Brackmann score did not fully communicate their facial function. Further, the single grade did not always correlate with the best or worst function along the four facial regions. The single House-Brackmann score most strongly correlated with the regional scoring of the eye (61%), followed by the nose/midface (40%), mouth (32%), and forehead (18%). The global score did not correlate with the worst regional score in 30 patients (79%). In 3 of 5 patients with synkinesis and an obligatory Grade 3 or higher in the global House-Brackmann grading system, the regional facial function was Grade 2 or better at one or more areas of the face. Conclusions In patients with differential facial function, a single global number is inadequate to describe facial function and primarily reflects the function of the eye. Regional assessment using the HBFNGS and reported as FwExNyMz more fully communicates facial function.

Auditory and vestibular symptoms and chronic subjective dizziness in patients with Ménière's disease, vestibular migraine, and Ménière's disease with concomitant vestibular migraine

Objective To compare presentations of Ménière’s disease (MD), vestibular migraine (VM), and Ménière’s disease plus vestibular migraine (MDVM), with and without comorbid chronic subjective dizziness (CSD). Study Design Retrospective review with diagnosis confirmed by consensus conference of investigators using published criteria for MD, VM, and CSD. Setting Ambulatory, tertiary dizziness clinic. Patients Approximately 147 consecutive patients with diagnoses of MD, VM, or MDVM, with/without comorbid CSD. Interventions Diagnostic consultation. Main Outcome Measures Similarities and differences between diagnostic groups in demographics; symptoms; and results of neurotologic, audiometric, and vestibular laboratory assessments. Results Seventy-six patients had MD, 55 MD alone. Ninety-two patients had VM, 71 VM alone. Twenty-one patients had MDVM, representing about one-quarter of those diagnosed with MD or VM. Clinical features thought to differentiate VM from MD were found in all groups. Twenty-seven patients with VM (38%) had ear complaints (subjective hearing loss, aural pressure, and tinnitus) during episodes of vestibular symptoms and headache, including 10 (37%) with unilateral symptoms. Conversely, 27 patients with MD alone (49%) had headaches with migraine features that did not meet full IHS diagnostic criteria, migrainous symptoms (photophobia, headache with vomiting), or first-degree relative with migraine. Including MDVM patients, 59% (45/76) of all patients with MD had migrainous features. Thirty-two patients had CSD; most (29; 91%) were in the VM group. Conclusion Comorbidity was common between MD and VM, and their symptoms overlapped. More specific diagnostic criteria are needed to differentiate these diseases and address their coexistence. CSD co-occurred with VM but was rarely seen with MD.

Long-term hearing outcomes following stereotactic radiosurgery for vestibular schwannoma: patterns of hearing loss and variables influencing audiometric decline

OBJECT The goals of this retrospective cohort study were as follows: 1) to describe the long-term prevalence and timing of hearing deterioration following low-dose (12- to 13-Gy marginal dose) stereotactic radiosurgery (SRS) for vestibular schwannoma (VS); and 2) to identify clinical variables associated with long-term preservation of useful hearing following treatment. METHODS Patients with serviceable hearing who underwent SRS for VS between 1997 and 2002 were studied. Data including radiosurgery treatment plans, tumor characteristics, pre- and posttreatment pure tone average, speech discrimination scores, and American Academy of Otolaryngology-Head and Neck Surgery hearing class were collected. Time to nonserviceable hearing was estimated using the Kaplan-Meier method. Univariate and multivariate associations with time to nonserviceable hearing were evaluated using Cox proportional hazards regression models. RESULTS Forty-four patients met the study criteria and were included. The median duration of audiometric follow-up was 9.3 years. Thirty-six patients developed nonserviceable hearing at a mean of 4.2 years following SRS. The Kaplan-Meier estimated rates of serviceable hearing at 1, 3, 5, 7, and 10 years following SRS were 80%, 55%, 48%, 38%, and 23%, respectively. Multivariate analysis revealed that pretreatment ipsilateral pure tone average (p < 0.001) and tumor size (p = 0.009) were statistically significantly associated with time to nonserviceable hearing. CONCLUSIONS Durable hearing preservation a decade after low-dose SRS for VS occurs in less than one-fourth of patients. Variables including preoperative hearing capacity and tumor size may be used to predict hearing outcomes following treatment. These findings may assist in pretreatment risk disclosure. Furthermore, these data demonstrate the importance of long-term follow-up when reporting audiometric outcomes following SRS for VS.