Faculty Bibliography

Background: The transcochlear approach extends the anterior exposure afforded by the translabyrinthine technique. Although this wider exposure allows improved access to cranial nerves and the brainstem with less retraction on the cerebellum, the classical description involving facial nerve transposition often results in permanent facial paresis. This study discusses the role of the transcochlear approach in resection of cerebellopontine angle tumors including patient selection, surgical technique, and outcomes. Study Design: Retrospective review. Methods: This is a retrospective review conducted at a single academic institution. Cases performed by our skull base team (neurotologist and neurosurgeon) between 2000 and 2015 were reviewed. All cases utilizing the transcochlear approach were included. Factors including tumor size, completeness of resection, facial nerve function, post-operative complications, and length of stay were analyzed. Results: Fourteen cases were included. All of these patients had pre-operative severe hearing loss except for two in which surgery was performed urgently in the setting of hydrocephalus and brainstem compression. Eight out of 14 patients had pre-operative facial paresis. Tumor size ranged from 2.2-7 cm in greatest dimension (mean = 4.56 cm). All patients underwent a transcochlear approach to and removal of tumor with blind sac of the external auditory canal. In addition, 3 patients underwent an immediate facial nerve to hypoglossal anastomosis. Post-operatively, patients remained in the hospital for 3-5 days (mean = 4). Of the patients who started out with normal facial nerve function (n = 6), 3 recovered to House-Brackmann scores II or greater. There were no reported CSF leaks requiring hospitalization and 1 abdominal hematoma from fat graft harvest. There was a single mortality reported in the peri-operative period; however, on autopsy the cause of death was unrelated to the surgery itself or any subsequent intracranial event. Conclusions: As in other surgical approaches destructive to the labyrinth, patients were selected with consideration of their pre-operative hearing status and/or their candidacy for hearing preservation surgery. Patients with pre-operative facial nerve paralysis and hearing loss were deemed particularly appropriate candidates for the transcochlear technique given the additional exposure and the lack of added morbidity. In these cases the surgeon also has the option to perform dynamic facial nerve reanimation at the time of tumor resection

OBJECTIVES: Although there are various available treatment options for unilateral severe-to-profound hearing loss, these options do not provide the benefits of binaural hearing since sound is directed from the poorer ear to the better ear. The purpose of this investigation was to review our center's experience with cochlear implantation in such patients in providing improved auditory benefits and useful binaural hearing. STUDY DESIGN: Retrospective chart review. METHODS: Twelve adult patients and four pediatric patients with unilateral severe-to-profound hearing loss received an implant in the poorer ear. Outcome measures performed preoperatively on each ear and binaurally included consonant-nucleus-consonant (CNC) monosyllabic words and sentences in noise. The mean pure-tune average in the better ear was within normal range. RESULTS: Test scores revealed a significant improvement in CNC and sentence in noise test scores from the preoperative to most recent postoperative evaluation in the isolated implant ear. All adult subjects use the device full-time. CONCLUSIONS: The data reveal significant improvement in speech perception performance in quiet and in noise in patients with single-sided deafness after implantation. Performance might depend on factors including length of hearing loss, age at implantation, and device usage.

Objective: Cerebral venous sinus thrombosis (CVST) is a rare but potentially dangerous complication following craniotomies involving the posterior fossa, the skull base, and craniotomies involving the superior sagittal sinus. Surgical manipulation of the sinuses has been shown to cause sinus thromboses of varying degrees. This phenomenon is often clinically silent, with only a small number of patients becoming symptomatic. Recent advances in neuroimaging provide higher resolution evaluation of cerebral sinuses pre and postoperatively, often revealing clinically silent filling defects. Although sinus thrombosis can be a major cause of morbidity and mortality, its incidence and factors that contribute to its formation in the postoperative population remain unclear. In addition, current guidelines advise to anticoagulate with Heparin upon diagnosis, which can be contraindicated for immediate post craniotomy patients. The goal of this study is to evaluate retrospective data of patient outcomes and factors that might contribute to sinus thrombus formation. Methods: A retrospective chart review was conducted at NYU Langone Medical Center. Ninety-seven patients were included who underwent either a retrosigmoid/translabrynthine/suboccipital resection of a posterior fossa tumor or a supratentorial craniotomy for resection of parasagittal/falcine tumor between July 1, 2014 and July 1, 2015. Pre operative Magnetic Resonance Venography (MRV) was obtained per the attending surgeon's discretion. Based on intraoperative findings, clinical symptoms, and surgeon's preference, a postoperative MRV was obtained. Decision to treat a thrombosis was made based on the results of the MRV and clinical symptoms. Treatments included observation, intravenous fluids (IVF) alone, anti-platelet therapy with Aspirin alone, or a combination of the two. Results: A total of 7 of 97 patients (7.22%) had postoperative sinus thrombosis. Of those patients, 5 had occlusion of the venous sinuses. In the occlusion group, 4 had preoperative imaging documenting patency of the sinuses. An additional 2 patients had postoperative MRVs revealing partial thrombosis of the sinus, 1 that was new and 1 that did not have a preoperative MRV for comparison. Compared with the cohort of patients without postoperative thromboses, there was no significant difference in age, BMI, length of surgery, or surgical approach. Of the 5 patients with postoperative thrombotic occlusion, 4 underwent intervention (1 with IVF alone, 2 with IVF and aspirin, and 1 with aspirin alone). Two patients with thromboses also developed CSF leaks requiring lumbar drainage and operative repair. One patient had a persistent CSF leak requiring a shunt. Of the 2 patients with partial thrombosis, 1 was placed on IVF and aspirin. At 3 months follow up, 1 out of 5 patients in the occlusion group had recanalization of the previously thrombosed sinus. Conclusions: MRV is a non-invasive method to evaluate the caliber and patency of dural venous sinuses in post craniotomy patients. Symptomatic thrombosis is rare and can be managed either conservatively or with IVF and/or anti platelet therapy, both which are safer than anticoagulation with heparin in post craniotomy patients. A larger prospective trial is necessary to further characterize the incidence of postoperative venous sinus thrombosis, identify risk factors, and to devise recommendations for therapy

OBJECTIVES/HYPOTHESIS: To evaluate the safety and efficacy of acoustic and electric sound processing for individuals with significant residual low-frequency hearing and severe-to-profound high-frequency sensorineural hearing loss. STUDY DESIGN: Prospective, single-arm repeated measures, single-subject design. METHODS: Fifty individuals, >/= 18 years old, with low-frequency hearing and severe high-frequency loss were implanted with the Cochlear Nucleus Hybrid L24 implant at 10 investigational sites. Preoperatively, subjects demonstrated consonant-nucleus-consonant word scores of 10% through 60% in the ear to be implanted. Subjects were assessed prospectively, preoperatively, and postoperatively on coprimary endpoints of consonant-nucleus-consonant words, AzBio sentences in noise, and self-assessment measures. RESULTS: Significant mean improvements were observed for coprimary endpoints: consonant-nucleus-consonant words (35.8 percentage points) and AzBio sentences in noise (32.0 percentage points), both at P < 0.001. Ninety-six percent of subjects performed equal or better on speech in quiet and 90% in noise. Eighty-two percent of subjects showed improved performance on speech in quiet and 74% in noise. Self-assessments were positive, corroborating speech perception results. CONCLUSION: The Nucleus Hybrid System provides significant improvements in speech intelligibility in quiet and noise for individuals with severe high-frequency loss and some low-frequency hearing. This device expands indications to hearing-impaired individuals who perform poorly with amplification due to bilateral high-frequency hearing loss and who previously were not implant candidates. LEVEL OF EVIDENCE: 2b. Laryngoscope, 2015.

OBJECTIVE: The aim of this case series was to assess the impact of auditory rehabilitation with cochlear implantation on the cognitive function of elderly patients over time. DESIGN: This is a longitudinal case series of prospective data assessing neurocognitive function and speech perception in an elderly cohort pre- and post-implantation. SETTING: University cochlear implant center. PARTICIPANTS: The patients were post-lingually deafened elderly female (mean, 73.6 years; SD, 5.82; range, 67-81 years) cochlear implant recipients (n=7). MEASUREMENTS: A neurocognitive battery of 20 tests assessing intellectual function, learning, short- and long-term memory, verbal fluency, attention, mental flexibility, and processing speed was performed prior to and 2-4.1 years (mean, 3.7) after cochlear implant (CI). Speech perception testing using Consonant-Nucleus-Consonant words was performed prior to implantation and at regular intervals postoperatively. Individual and aggregate differences in cognitive function pre- and post-CI were estimated. Logistic regression with cluster adjustment was used to estimate the association (%improvement or %decline) between speech understanding and years from implantation at 1 year, 2 years, and 3 years post-CI. RESULTS: Improvements after CI were observed in 14 (70%) of all subtests administered. Declines occurred in five (25%) subtests. In 55 individual tests (43%), post-CI performance improved compared to a patient's own performance before implantation. Of these, nine (45%) showed moderate or pronounced improvement. Overall, improvements were largest in the verbal and memory domains. Logistic regression demonstrated a significant relationship between speech perception and cognitive function over time. Five neurocognitive tests were predictive of improved speech perception following implantation. CONCLUSION: Comprehensive neurocognitive testing of elderly women demonstrated areas of improvement in cognitive function and auditory perception following cochlear implantation. Multiple neurocognitive tests were strongly associated with current speech perception measures. While these data shed light on the complex relationship between hearing and cognition by showing that CI may slow the expected age-related cognitive decline, further research is needed to examine the impact of hearing rehabilitation on cognitive decline.

OBJECTIVE AND IMPORTANCE: Preoperative chronic otitis media (COM) is a risk factor for postoperative infection after cochlear implantation (CI), but its management varies by surgeon. Our case highlights a strategy for implant preservation in a patient with a history of recurrent cochlear implant infection. CLINICAL PRESENTATION: A 70-year-old woman with a history of chronic lymphocytic leukemia presented in 2005 with bilateral COM and sensorineural hearing loss meeting CI candidacy. Four months after left mastoid obliteration with abdominal fat graft and external auditory canal closure, a left CI was placed. Subsequent postauricular cellulitis resolved with oral antibiotics. A similar two-stage CI was performed on the right without complication. During the following year, numerous left-sided infections and fluid collections developed but were treated unsuccessfully with intravenous (IV) antibiotics and operative debridement. With concern for biofilm colonization, the implant was explanted and the electrode left in the cochlea. After reimplantation in 2010, infections resumed despite long-term IV antibiotics and incision and drainage. Intervention and technique In 2012, the left mastoid cavity was exteriorized and converted to standard canal wall-down anatomy. Bone pate was placed over the electrode, followed by cadaveric acellular dermis and a split-thickness skin graft. After more than 2 years, her better-performing CI remains infection-free. CONCLUSION: After 6 years of postoperative infections unresponsive to aggressive medical management, surgical interventions, and period of device removal, our patient's infections resolved after mastoid exteriorization and multilayered protection of the electrode. This strategy may enable implant preservation in patients with recurrent post-CI infection in an obliterated cavity.

Hearing impairment is a significant disability. According to the World Health Organization (WHO), more than 80% of the world's approximately 120 million people with hearing impairment live in developing countries. Cochlear implant is the only therapeutic intervention for those with severe-profound sensorineural hearing loss. We are reporting an interesting case of the very first cochlear implant operation carried out in Uganda. The patient was a 23 year old male whose presenting complaint was inability to hear in the left ear for three and a half years and in the right ear for one year. He had been treated for TB(Tuberculosis) mastoiditis. After the 8 months of treatment, the otorrhea persisted and he underwent a tympanomastoidectomy on the same ear. He reported no familial history of hearing loss. On examination, ENT examination revealed a small pars flaccida retration pocket of the right tympanic membrane with cholesteatoma. The left ear had an intact tympanic membrane. Pure tone audiometry revealed profound sensorineural hearing loss in both ears (see attached PTA results), CT scan of the temporal bone showed normal inner ear anatomy bilaterally and mild sclerotic changes in both mastoid bones. He then had surgery on his right ear which included cochlear implantation. The cochlear implant (CI) was activated on the first postoperative day remotely via internet with the help of the cochlear implant team at New York University Cochlear Implant Center and the patient was immediately able to appreciate some sounds. He received a pneumococcal vaccine on the first postoperative day and was discharged the following day.

OBJECTIVE: The aim of this study was to develop a multidimensional metric for assessing quality of life (QoL) in patients with neurofibromatosis type 2 (NF2). STUDY DESIGN: Electronically distributed questionnaire. SETTING: University tertiary care hospital, NF2 support groups. SUBJECTS AND METHODS: Structured interviews with NF2 providers and patients identified relevant domains. Items in each domain were extracted from validated QoL modules, then combined with items unique to NF2 and pretested on NF2 providers and patients. The final 61-item questionnaire was administered electronically to patients with NF2 (N = 118). The form assessed overall QoL and 11 additional domains, including hearing, balance, facial function, vision, oral intake, future uncertainty, psychosocial, cognition, sexual activity, pain, and vocal communication. Responses were compared with reference values for the general population, patients with head and neck cancer, and patients with brain cancer. RESULTS: Overall, QoL in patients with NF2 was lower than that of the general population (P < .01) and similar to that of patients with cancer. Patients with more facial weakness, hearing loss, and imbalance reported significantly lower QoL. However, domains most predictive of overall QoL were psychosocial, future uncertainty, and pain. Compared with patients with head and neck and brain cancer, patients with NF2 demonstrated significantly higher levels of psychosocial stressors, including disease-related anxiety, personal and financial stress, and lack of social support (P < .01). CONCLUSION: Psychosocial stress and pain significantly affect QoL in NF2, indicating that mental health, pain management, and financial counseling could have an important impact on QoL in this population.

OBJECTIVES: X-linked deafness is a potential etiology of sensorineural hearing loss characterized by bulbous dilatation of the fundus of the internal auditory canal (IAC) and the absence of the bony plates separating the basal turn of the cochlea and IAC. These malformations predispose patients to IAC insertion during cochlear implantation (CI). Our objective is to describe the surgical technique, audiometric and speech performance outcomes in a group of patients with this unique cochlear malformation. METHODS: A retrospective chart review was performed of all patients at a tertiary care facility who underwent CI between January 2006 and July 2011. RESULTS: A total of five patients were identified with radiographic findings characteristic of X-linked deafness, specifically a deficient modiolous, absent lamina cribrosa and bulbous IAC. A modified cochleostomy was utilized to ensure electrode insertion within the scala tympani avoiding the IAC. In each case, fluoroscopy was utilized to visual electrode progression during insertion and complete insertion was accomplished. All patients demonstrated improved speech performance following implantation. CONCLUSIONS: Utilizing the techniques described, patients with these unique radiographic findings consistent with X-linked deafness may successfully undergo CI with excellent potential for auditory rehabilitation.