Vincent Y. W. Lin

Combined Corticosteroid and Antiviral Treatment for Bell Palsy: A Systematic Review and Meta-analysis

CONTEXTnNew evidence has emerged regarding the use of corticosteroids and antiviral agents in Bell palsy.nnnOBJECTIVEnTo estimate the association of corticosteroids and antiviral agents with the risk of unsatisfactory facial recovery in patients with Bell palsy.nnnDATA SOURCESnThe search included MEDLINE, EMBASE, CENTRAL, PsychInfo, CINAHL, Web of Science, PAPERSFIRST, PROCEEDINGSFIRST, and PROQUEST to identify studies up to March 1, 2009.nnnSTUDY SELECTION AND DATA EXTRACTIONnEligible studies were randomized controlled trials comparing treatment with either corticosteroids or antiviral agents with a control and measuring at least 1 of the following outcomes: unsatisfactory facial recovery (> or = 4 months), unsatisfactory short-term recovery (6 weeks to < 4 months), synkinesis and autonomic dysfunction, or adverse effects. Two reviewers extracted data on study characteristics, methods, and outcomes. Disagreement was resolved by consensus.nnnRESULTSnEighteen trials involving 2786 patients were eligible. Regression analysis identified a synergistic effect when corticosteroids and antiviral agents were administered in combination compared with alone (odds ratio for interaction term, 0.54 [95% confidence interval {CI}, 0.35-0.83]; P = .004). Meta-analysis using a random-effects model showed corticosteroids alone were associated with a reduced risk of unsatisfactory recovery (relative risk [RR], 0.69 [95% CI, 0.55-0.87]; P = .001) (number needed to treat to benefit 1 person, 11 [95% CI, 8-25]), a reduced risk of synkinesis and autonomic dysfunction (RR, 0.48 [95% CI, 0.36-0.65]; P < .001) (number needed to treat to benefit 1 person, 7 [95% CI, 6-10]), and no increase in adverse effects. Antiviral agents alone were not associated with a reduced risk of unsatisfactory recovery (RR, 1.14 [95% CI, 0.80-1.62]; P = .48). When combined with antiviral agents, corticosteroids were associated with greater benefit (RR, 0.48 [95% CI, 0.29-0.79]; P = .004) than antiviral agents alone. When combined with corticosteroids, antiviral agents were associated with greater risk reduction of borderline significance compared with corticosteroids alone (RR, 0.75 [95% CI, 0.56-1.00]; P = .05).nnnCONCLUSIONSnIn Bell palsy, corticosteroids are associated with a reduced risk of unsatisfactory recovery. Antiviral agents, when administered with corticosteroids, may be associated with additional benefit.

Inhibition Of Notch Activity Promotes Nonmitotic Regeneration of Hair Cells in the Adult Mouse Utricles

The capacity of adult mammals to regenerate sensory hair cells is not well defined. To explore early steps in this process, we examined reactivation of a transiently expressed developmental gene, Atoh1, in adult mouse utricles after neomycin-induced hair cell death in culture. Using an adenoviral reporter for Atoh1 enhancer, we found that Atoh1 transcription is activated in some hair cell progenitors (supporting cells) 3 d after neomycin treatment. By 18 d after neomycin, the number of cells with Atoh1 transcriptional activity increased significantly, but few cells acquired hair cell features (i.e., accumulated ATOH1 or myosin VIIa protein or developed stereocilia). Treatment with DAPT, an inhibitor of γ-secretase, reduced notch pathway activity, enhanced Atoh1 transcriptional activity, and dramatically increased the number of Atoh1-expressing cells with hair cell features, but only in the striolar/juxtastriolar region. Similar effects were seen with TAPI-1, an inhibitor of another enzyme required for notch activity (TACE). Division of supporting cells was rare in any control or DAPT-treated utricles. This study shows that mature mammals have a natural capacity to initiate vestibular hair cell regeneration and suggests that regional notch activity is a significant inhibitor of direct transdifferentiation of supporting cells into hair cells following damage.

Is a plexiform neurofibroma pathognomonic of neurofibromatosis type I

Objectives/Hypothesis: Several prominent textbooks have claimed that a plexiform neurofibroma is pathognomonic for neurofibromatosis type I. This is not in agreement with the National Institutes of Health criteria, which require two signs to be present, one of which can be a plexiform neurofibroma. Is a plexiform neurofibroma pathognomonic for neurofibromatosis type I?

Unilateral Acoustic Neuromas: Long-Term Hearing Results in Patients Managed with Fractionated Stereotactic Radiotherapy, Hearing Preservation Surgery, and Expectantly

Introduction: Hearing preservation is invariably a consideration when exploring treatment options for acoustic neuromas. We reviewed the long‐term hearing results of patients who were treated using 1) hyperfractionated stereotactic radiotherapy (HSR), 2) hearing preservation tumor excision surgery (HPTES), and 3) expectantly (no treatment).

Postlingually deaf adults of all ages derive equal benefits from unilateral multichannel cochlear implant.

BACKGROUNDnControversy still exists regarding the impact of age on speech recognition following cochlear implant in postlingually deaf adults. In some studies elderly recipients did not perform as well as younger patients on standard speech recognition tests. Furthermore, previous studies have shown that cochlear implantation improves quality of life, as measured by self-administered questionnaires, but the sample sizes of these studies have been relatively small, thus making age stratification a challenge.nnnPURPOSEnThe primary objective was to assess whether the age at which a patient receives a unilateral cochlear implant affects improvements in speech recognition scores and perceived quality of life. A secondary objective was to determine whether preoperative use of hearing aids correlates with improvement in speech recognition and perceived quality of life after cochlear implantation.nnnRESEARCH DESIGNnA retrospective study in a tertiary referral center.nnnPATIENTSnA total of 161 postlingually deaf adults, who were divided based on age (<50, 50-65, >65) and on prior hearing aid(s) use.nnnINTERVENTIONnAll patients received a unilateral multichannel cochlear implant.nnnDATA COLLECTION AND ANALYSISnSpeech recognition was quantified by percent correct scores on the Hearing in Noise Test sentences delivered in a quiet setting only (HINT%), and quality of life was quantified by the Hearing Handicap Inventory (HHI) before and 1 yr after cochlear implantation.nnnRESULTSnSpeech recognition, as measured by HINT%, improved significantly and to similar extents in all three age groups following cochlear implantation. Similarly, quality of life as quantified by HHI improved markedly and to similar extents in all age groups. Whether hearing aids were used pre-implant, or whether the cochlear implant (CI) was implanted on the same side or contralateral to the hearing aid side, had no substantial effect on the patients performances on either speech recognition or quality of life. Moreover, there were no statistically significant correlations between pre-implant speech recognition scores and pre-implant quality of life scores or between postimplant speech recognition scores and postimplant quality of life scores.nnnCONCLUSIONnThe findings of the present study demonstrate that cochlear implantation improves HINT% and HHI scores to similar extents across all age groups. This finding suggests that elderly patients may derive speech recognition and quality of life benefits similar to those of younger patients and that age should not be an essential factor in the determination of CI candidacy. Furthermore, prior use of a hearing aid, and its location in relation to the cochlear implant, does not influence the extent of improvement in speech recognition or quality of life measurements following cochlear implantation.

Unilateral multi-channel cochlear implantation results in significant improvement in quality of life.

Objectives To investigate the effects of unilateral multi-channel cochlear implant surgery on health-related quality of life and to determine if there is an age-related impact of cochlear implantation on these effects. Design Prospective study. Setting Tertiary health-care center. Methods The Short Form-36 survey (SF-36) was administered to determine the health-related quality of life of 283 age-stratified patients before and after cochlear implant surgery. Main Outomes Precochlear to postcochlear implantation changes in health-related quality of life as determined by the SF-36 questionnaire. Results There were significant increases in precochlear and postcochlear implantation scores for 5 of the 8 SF-36 survey domains: vitality, physical role functioning, mental health, emotional role functioning, and social functioning. Significant differences were found between age groups in the domains of social functioning, emotion role functioning, and mental health. Conclusion Cochlear implant surgery significantly improves health-related quality of life as categorically stratified by the SF-36 questionnaire. These improvements were most evident in the mental health, emotional and social functioning, and physical functioning at work questions of the survey. Cochlear implant recipients younger than 65 years perceive a greater improvement in their level of energy, mental health, and social function compared with those older than 65 years. Level of Evidence N/A.

Global assessment of outcomes after varying reinnervation techniques for patients with facial paralysis subsequent to acoustic neuroma excision.

Objective: To determine whether there are objective and/or subjective differences in facial function, tongue function and quality of life in patients who have undergone 1) direct facial-to-facial (Primary, 4 patients), 2) direct facial-to-hypoglossal (End-to-End, 7 patients), 3) end-to-side facial-to-hypoglossal nerve anastomoses with sural nerve/greater auricular nerve interposition (End-to-Side with Interposition Graft, 7 patients), and 4) end-to-side facial-to-hypoglossal nerve anastomoses (End-to-Side, 7 patients) after acoustic neuroma excision. Setting: Tertiary university referral center. Study Design: Case series. Methods: Twenty-five patients with complete facial paralysis after acoustic neuroma surgery who have also undergone 1 of 4 types of facial musculature reinnervation volunteered for the study. The mean duration of follow-up was 11.5 years (range, 1-25 yr). Each patient was globally assessed using the Sunnybrook Facial Grading scale (objective), Facial Disability Index (subjective), Oral-Pharyngeal Disability Index (subjective), Tongue Movement/Muscle symmetry (objective), and short-form SF-36 quality-of-life index (subjective). Results: Patients who underwent direct facial-to-hypoglossal anastomosis had generally poorer outcomes some of which were statistically significant (p < 0.05) in some components of the Oral-Pharyngeal Disability Index. All of these patients had reduced lateral tongue movement as well as ipsilateral tongue atrophy. There was no significant difference in the Sunnybrook Facial Grading scale scores in those patients in whom end-to-side interposition reinnervation surgery was performed compared with those in whom direct end-to-side anastomosis surgery was performed. Conclusion: Complete sacrifice of the ipsilateral hypoglossal nerve does present with clinically and statistically significant morbidity in patients. Facial function is statistically similar in patients who underwent an end-to-side anastomosis with or without an interposition graft. These findings suggest thatin the setting of the posttranslabryinthine approach, the technicallyeasier interposition graft procedure is not acompromise.

A novel method in predicting immediate postoperative facial nerve function post acoustic neuroma excision.

Abstract: To determine whether the percentage calculated by dividing the amplitude of postexcision direct facial nerve stimulus responses (at pontomedullary junction) by the amplitude of distal ipsilateral transcutaneous (stylomastoid region) maximal stimulus responses and response amplitude progression by increasing stimulus intensities have predictive value for determining normal or near-normal (House-Brackmann Grade 1 or 2) immediate postoperative facial nerve function. Study Design: Intraoperative recordings of three muscle groups: 1) frontalis, 2) orbicularis oculi, and 3) orbicularis oris. Postexcision direct facial nerve stimulation at the pontomedullary junction and transcutaneous maximal facial nerve stimulation at the ipsilateral stylomastoid region and their associated response amplitudes were recorded. Setting: Tertiary referral center. Patients and Methods: Patients who underwent acoustic neuroma surgery from January 2004 to March 2006 with intraoperative facial nerve monitoring and an intact facial nerve after tumor excision were included. Recordings were available for 38 patients. Results: With a stimulus intensity of 0.3 mA at the root exit zone, there was an 81% positive predictive value in patients that exhibited a compound action potential of greater than 20% of maximum (sensitivity, 81%). This increased to 93% when the compound action potential was greater than 50% of maximum. When the amplitude increase was greater than 5 &mgr;V, there was a 77% positive predictive value (sensitivity, 87%). Conclusion: The percentage of the response amplitude of direct facial nerve stimulation at the pontomedullary junction when compared with the maximum response amplitude of ipsilateral transcutaneous stimulation at the stylomastoid foramen is a good predictor of normal to near-normal immediate postoperative facial nerve function. Progression of amplitude response also seems to be a good predictor of normal to near-normal immediate postoperative facial nerve function.

Expanded selection criteria in adult cochlear implantation.

Abstract Cochlear implantation has become the standard procedure for restoring substantial hearing in the profoundly deaf. The excellent performance of most of the CI recipients coupled with the rapid evolution of implant technology lead to a distinct expansion in selection criteria for CI. These changes in candidacy primarily include patients with (1) moderate preoperative speech recognition with hearing aids, (2) significant residual hearing, (3) single-sided deafness, and (4) geriatric patients. Many of these patients’ conditions were regarded as a clear contraindication to CI only a few years ago. In this article an overview of the current and new aspects of candidacy for cochlear implantation is provided.

Results with cochlear implantation in adults with speech recognition scores exceeding current criteria.

Objectives The primary purpose of this study was to evaluate a group of postlingually deafened adults, whose aided speech recognition exceeded commonly accepted candidacy criteria for implantation. The study aimed to define performance and qualitative outcomes of cochlear implants in these individuals compared with their optimally fitted hearing aid(s). Study Design Retrospective case series. Setting Tertiary referral center. Patients All postlingually deafened subjects (N = 27), who were unsuccessful hearing aid users implanted between 2000 and 2010 with a preimplantation Hearing in Noise Test (HINT) score of 60% or more were included. Intervention We compared patients’ preoperative performance (HINT score) with hearing aids to postoperative performance with the cochlear implant after 12 months of device use. In addition, the Hearing Handicap Inventory questionnaire was used to quantify the hearing-related handicap change perceived after the implantation. Results The study group demonstrated significant postoperative improvement on all outcome measures; most notably, the mean HINT score improved from 68.4% (standard deviation, 8.3) to 91.9% (standard deviation, 9.7). Additionally, there was a significant improvement in hearing-related handicap perceived by all patients. Conclusion The envelope of implantation candidacy criteria continues to expand as shown by this study’s cohort. Patient satisfaction and speech recognition results are very encouraging in support of treating those who currently perform at a level above the conventional candidacy threshold but struggle with optimally fitted hearing aids.