Repeat Radiosurgery for Sporadic Vestibular Schwannoma After Primary Radiosurgical Failure: An International Multi-institutional Investigation.

OBJECTIVE: To describe outcomes of patients with sporadic vestibular schwannoma (VS) who underwent repeat stereotactic radiosurgery (SRS) after primary SRS failure. STUDY DESIGN: Multi-institutional historical cohort study. SETTING: Five tertiary care referral centers. PATIENTS: Adults ≥18 years old with sporadic VS. INTERVENTION: Primary and repeat treatment with SRS. MAIN OUTCOME MEASURE: Microsurgery-free survival after repeat SRS. RESULTS: Across institutions, 32 patients underwent repeat SRS after primary SRS. Most patients (74%) had tumors with cerebellopontine angle extension at primary SRS (median size, 13.5 mm [interquartile range, 7.5-18.8] mm). After primary SRS, patients underwent repeat SRS at a median of 4.8 years (interquartile range, 3.2-5.7 yr). For treatment modality, 30 (94%) patients received gamma knife for primary treatment and 31 (97%) patients received gamma knife as their repeat treatment. Median tumor volume increased from 0.970 cm3 at primary SRS to 2.200 cm3 at repeat SRS. Facial nerve function worsened in two patients after primary SRS and in two patients after repeat SRS. There were no instances of intracranial complications after repeat SRS. Microsurgery-free survival rates (95% confidence interval; number still at risk) at 1, 3, and 5 years after repeat SRS were 97% (90-100%, 24), 84% (71-100%, 13), and 68% (48-96%, 6), respectively. There was one occurrence of malignancy diagnosed after repeat radiosurgery. CONCLUSION: Overall, repeat SRS for sporadic VS has comparable risk profile, but lower rates of tumor control, compared with primary SRS.

Neurofibromatosis Type II and Facial Paralysis: Clinical Evaluation and Management.

BACKGROUND: Facial paralysis secondary to neurofibromatosis type 2 (NF2) presents the reconstructive surgeon with unique challenges because of its pathognomonic feature of bilateral acoustic neuromas, involvement of multiple cranial nerves, use of antineoplastic agents, and management. Facial reanimation literature on managing this patient population is scant. METHODS: A comprehensive literature review was performed. All patients with NF2-related facial paralysis who presented in the past 13 years were reviewed retrospectively for type and degree of paralysis, NF2 sequelae, number of cranial nerves involved, interventional modalities, and surgical notes. RESULTS: Twelve patients with NF2-related facial paralysis were identified. All patients presented after resection of vestibular schwannoma. Mean duration of weakness before surgical intervention was 8 months. On presentation, one patient had bilateral facial weakness, 11 had multiple cranial nerve involvement, and seven were treated with antineoplastic agents. Two patients underwent gracilis free functional muscle transfer, five underwent masseteric-to-facial nerve transfer (of whom two were dually innervated with a crossfacial nerve graft), and one patient underwent depressor anguli oris myectomy. Trigeminal schwannomas did not affect reconstructive outcomes if trigeminal nerve motor function on clinical examination was normal. In addition, antineoplastic agents such as bevacizumab and temsirolimus did not affect outcomes if stopped in the perioperative period. CONCLUSIONS: Effectively managing patients with NF2-related facial paralysis necessitates understanding the progressive and systemic nature of the disease, bilateral facial nerve and multiple cranial nerve involvement, and common antineoplastic treatments. Neither antineoplastic agents nor trigeminal nerve schwannomas associated with normal examination affected outcomes. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, V.

Audiometric and Patient-Reported Outcomes in Single-Sided Deafness Cochlear Implant Recipients Using the CIQOL-35.

OBJECTIVE: Describe the effect that cochlear implantation (CI) has on audiometric outcomes and quality of life (QOL) in patients with single-sided deafness (SSD). STUDY DESIGN: Retrospective case review. SETTING: Tertiary university hospital system. METHODS: Preoperative and postoperative AzBio performance and Cochlear Implant Quality of Life-35 (CIQOL-35) Profile scores in CI patients with SSD were compared, and postoperative measures were compared to those from CI patients without SSD. RESULTS: Seventeen patients with unilateral CI and contralateral unaided pure-tone averages ≤30 dB were included. The median age was 60.2 (interquartile range [IQR], 50.9-64.9 years), and 7/17 (41%) were women. Median daily use was 8.2 hour (IQR, 5.4-11.9 h). The median preoperative AzBio quiet score in the ear to be implanted was 3% (IQR, 0%-6%). After a median follow-up of 12.0 months, the median postoperative AzBio quiet score was 76% (IQR, 47%-86%) (p < .01). SSD subjects demonstrated statistically significant improvements in median scores on the following CIQOL-35 subdomains following implantation: Entertainment (17 preoperatively vs 21 postoperatively), Listening Effort (12 vs 14), Social (17 vs 22), and Global (28 vs 35; p < .05). SSD patients achieved equal or higher postoperative CIQOL-35 scores in most subdomains (6/7) compared to an age-matched group of non-SSD CI recipients who underwent unilaterally (N = 19) or sequential (N = 6) implantation. CONCLUSION: SSD CI patients not only demonstrate significant improvements in speech perception testing in the implanted ear but also exhibit improvement in multiple QOL subdomains on the CIQOL-35, the only validated cochlear implant QOL instrument.

Hearing Preservation After Intervention in Vestibular Schwannoma.

OBJECTIVE: This study aimed to assess the durability of audiological outcomes after radiation and surgery in the management of vestibular schwannoma. STUDY DESIGN: Retrospective review. SETTING: Tertiary academic center. PATIENTS: Adults with sporadic vestibular schwannoma and serviceable hearing at the time of intervention. INTERVENTIONS: Gamma Knife, middle cranial fossa, or retrosigmoid approaches. MAIN OUTCOME MEASURES: Pure-tone audiometry and speech discrimination scores. RESULTS: Postintervention serviceable hearing (class A/B) was preserved in 70.4% (n = 130; mean follow-up, 3.31 yr; range, 0-15.25 yr). Of the 49 patients treated with radiation, 19 (39.6%) had serviceable hearing at last follow-up, compared with 38 (46.9% of 81) who underwent retrosigmoid (n = 36 [44.4%]) and middle cranial fossa (n = 45 [55.6%]) approaches (odds ratio [OR], 1.40; 95% confidence interval [CI], 0.67-2.82; p = 0.47). A matched analysis by age, tumor volume, and preintervention hearing (n = 38) also found no difference in hearing preservation (HP) likelihood between surgery and radiation (OR, 2.33; 95% CI, 0.24-35.91; p = 0.59). After initial HP, 4 (9.5%) surgical versus 10 (37.0%) radiated patients subsequently lost residual serviceable (A/B) hearing (OR, 0.18; 95% CI, 0.06-0.69; p = 0.01) at a mean 3.74 ± 3.58 and 4.73 ± 3.83 years after surgery and radiation, respectively. Overall, 5- and 10-year HP rates (A/B) after initially successful HP surgery were 84.4 and 63.0%, respectively. However, survival estimates declined to 48.9% at 5 years and 32.7% at 10 years when patients with immediate postoperative serviceable hearing loss were also included, which were comparable to radiation-HP rates at 5 and 10 years of 28.0 and 14.2%, respectively ( p = 0.75). CONCLUSIONS: After vestibular schwannoma intervention, overall HP was similar between radiated and surgical cohorts. However, when successful, surgical approaches offered more durable hearing outcomes at long-term follow-up.

COVID-19 and ear endoscopy in otologic practices.

PURPOSE: Otolaryngologists have had to postpone the majority of surgical procedures in the current COVID-19 pandemic. Airborne transmission, beyond the projection of droplets from upper airways, expose healthcare workers to a risk of viral infection. Aerosol generating procedures (AGP) increase the risk of viral transmission to staff within the operating room. METHODS: Surgery of middle ear and mastoid is also considered an AGP, particularly mastoidectomy performed using a high-speed drill. The authors report their experience in endoscopic ear surgery as an alternative technique to reduce AGP in otologic procedures. RESULTS: Transcanal endoscopic ear surgery is a reliable technique used to manage many otologic conditions. CONCLUSION: The endoscopic approach may reduce the risk of viral transmission to operating room staff by reducing the need for mastoidectomy.

Facial Nerve Trauma: Clinical Evaluation and Management Strategies.

The field of facial paralysis requires the reconstructive surgeon to apply a wide spectrum of reconstructive and aesthetic principles, using a comprehensive array of surgical tools, including microsurgery, peripheral nerve surgery, and aesthetic facial surgery on the road to optimize patient outcomes. The distinct deficits created by different anatomical levels of facial nerve injury require a fundamental understanding of facial nerve anatomy. Palsy duration, followed by location and mechanism, will determine mimetic muscle salvageability, by means of either direct repair, grafting, or nerve transfers, whereas longer palsy durations will necessitate introducing a new neuromuscular unit, whether by muscle transfer or free functional muscle transplant. A thorough history, physical examination, and basic understanding of ancillary studies, emphasizing palsy duration, location, and mechanism of injury, are critical in evaluation, prognostication, and treatment strategies in traumatic facial palsy patients. The importance of ancillary and aesthetic procedures cannot be overstated. Although these do not provide motion, they constitute essential tools in the treatment of facial paralysis, providing both protective and improved aesthetic outcomes, yielding the highest impact in final surgeon and patient satisfaction, bringing our patients to smile not only on the outside, but also on the inside.

Clinical outcomes following cochlear implantation in children with inner ear anomalies.

OBJECTIVE: A significant proportion of children with congenital hearing loss who are candidates for cochlear implants (CIs) may have inner ear malformations (IEMs). Surgical and speech outcomes following CI in these children have not been widely reported. METHODS: The charts of children who were evaluated for a CI between 1/1/1986 and 12/31/2014 at a university-based tertiary level pediatric cochlear implant center were reviewed. Principal inclusion criteria included (i) age 1-18 years, (ii) history of bilateral severe to profound sensorineural hearing loss, and (iii) limited benefit from binaural amplification. Exclusion criteria included (i) underlying diagnosis of neurodevelopmental disorder and (ii) lack of follow up for speech assessment if a CI was performed. The following outcome measures were reviewed: (i) imaging findings with magnetic resonance imaging or high resolution computed tomography, (ii) intraoperative complications, and (iii) speech perception categorized as the ability to perceive closed set, open set, or none. RESULTS: The prevalence of IEMs was 27% (102 of 381), of which 79% were bilateral. Cochlear dysplasia accounted for 30% (40 of 136) of the anomalies. Seventy-eight of the 102 patients received a CI (78%). Surgery was noted to be challenging in 24% (19 of 78), with a perilymphatic gusher being the most common intraoperative finding. Cochlear dysplasia, vestibular dysplasia and cochlear nerve hypoplasia were associated with poor speech perception (open OR closed set speech recognition scores, 0-23%), although the outcomes in children with enlarged vestibular aqueduct were similar to those of children with normal inner ear anatomy (65%). CONCLUSIONS: Cochlear implantation is safe in children with IEMs. However, the speech perception outcomes are notably below those of patients with normal anatomy, with the exception of when an enlarged vestibular aqueduct is present.

Neuropsychological testing in the screening for cochlear implant candidacy.

OBJECTIVE: To demonstrate the utility of neuropsychological assessment in the screening process for pediatric cochlear implant candidacy. STUDY DESIGN: Prospective and ongoing evaluation of children with profound bilateral hearing loss using age-specific neuropsychological test batteries. METHODS: Eighteen children who met audiological criteria for cochlear implantation were evaluated by two age-specific neuropsychological tests. The Vineland Adaptive Behavior Scales survey assesses several domains of behavioral functions (communication, daily living skills, socialization, and gross motor skills). The Mullen Scales of Early Learning assesses the child's visual perception, speech and language, and motor abilities. The Leiter International Performance Scale-Revised assesses intellectual ability. RESULTS: All patients underwent the Vineland Adaptive Behavior Scales survey. Overall scores were lower than normative means with a mean composite score in the 7th percentile. In addition, there was a strong inverse correlation between score and age of testing. Ten children were assessed using the Mullen Scales of Early Learning, and, again, there was a strong inverse correlation between score and age of testing. Intellectual ability was assessed in seven children using the Leiter International Performance Scale-Revised and was found to be lower than normative means with a mean score in the 13th percentile. CONCLUSIONS: Neuropsychological testing of profoundly deaf children provides a detailed and accurate assessment of the child's cognitive, behavioral, and motor functions. The profoundly deaf child does not develop at the same rate as normal children in cognitive and behavioral domains. Neuropsychological testing is a useful tool for screening for cochlear implant candidacy and has the potential to track changes before and after implantation.

Neuropsychological testing in the screening for cochlear implant candidacy.

OBJECTIVE: To demonstrate the utility of neuropsychological assessment in the screening process for pediatric cochlear implant candidacy. STUDY DESIGN: Prospective and ongoing evaluation of children with profound bilateral hearing loss using age-specific neuropsychological test batteries. METHODS: Eighteen children who met audiological criteria for cochlear implantation were evaluated by two age-specific neuropsychological tests. The Vineland Adaptive Behavior Scales survey assesses several domains of behavioral functions (communication, daily living skills, socialization, and gross motor skills). The Mullen Scales of Early Learning assess the child's visual perception, speech and language, and motor abilities. The Leiter International Performance Scale-Revised assesses intellectual ability. RESULTS: All patients underwent the Vineland Adaptive Behavior Scales survey. Overall scores were lower than normative means with a mean composite score in the 7th percentile. In addition, there was a strong inverse correlation between score and age of testing. Ten children were assessed using the Mullen Scales of Early Learning, and, again, there was a strong inverse correlation between score and age of testing. Intellectual ability was assessed in seven children using the Leiter International Performance Scale-Revised and was found to be lower than normative means with a mean score in the 13th percentile. CONCLUSIONS: Neuropsychological testing of profoundly deaf children provides a detailed and accurate assessment of the child's cognitive, behavioral, and motor functions. The profoundly deaf child does not develop at the same rate as normal children in cognitive and behavioral domains. Neuropsychological testing is a useful tool for screening for cochlear implant candidacy and has the potential to track changes before and after implantation.