Faculty Bibliography

Background: Salvage surgery for progressive symptoms or recurrent growth of vestibular schwannoma (VS) after stereotactic radiosurgery (SRS) is uncommon. It has been reported to be more difficult, the same or easier than expected compared with microsurgical resection (MS) of treatment-naive VS, with variable facial nerve outcomes. We conducted a matched cohort analysis to evaluate facial nerve outcomes for patients undergoing MS for progressive symptoms or recurrent growth of VS after SRS compared with MS for treatment-naive VS. Methods: We evaluated the records of 365 non-NF2 VS patients treated with MS from 2001 to 2014. All patients who had postoperative notes were considered to have "adequate follow-up" and included in the study. Nine patients underwent resection for VS after prior SRS (MS-POST SRS) with no prior intervention, 7 of which had adequate follow-up, and 331 patients that underwent MS for treatment naive VS (MS-NO SRS), 317 of which had adequate follow-up. From this dataset, we created propensity score matched cohorts to retrospectively compare facial nerve preservation and clinical outcomes. The propensity score model incorporated age at surgery, tumor size, and pre-operative HB grade, and cases were matched at a ratio of 4:1 MS-NO SRS to MS-POST SRS cases. Results: The MS-POST SRS (n = 7) and MS-NO SRS (n = 28) groups differed only in preoperative hearing status (p < 0.01). Preoperative HB grades were similar between MS-POST SRS and MS-NO SRS groups (p>0.3). The average time-to-intervention after SRS in the MS-POST SRS group was 44.4 months. The indication for MS after SRS was symptom progression with tumor growth in 4 cases, and only tumor growth in the remaining 3. Postoperatively, 3 cases in the MS-POST SRS group demonstrated improvement in HB grade and none became worse whereas 3 of 28 cases in the MS-NO SRS group demonstrated improvement and 12 cases had decrement in HB grade relative to baseline (p < 0.05). There was no significant difference in the extent of resection with both cohorts (MS-POST SRS and MS-NO SRS) attaining gross total resection in 85.7% of cases. Subsequent intervention was not required for any case in the MS-POST SRS group and 2 cases in the MS-NO SRS group, although this difference was not significant. Both cases requiring subsequent intervention in the MS-POST SRS group were gross total resections via the translabyrinthine approach, with subsequent intervention required at 62 and 48 months. There were no significant differences in the complication rate for individual complications or the overall complication rate between cohorts. Conclusions: At a center with high clinical volume, microsurgical resection of VS after prior SRS was associated with good postoperative facial nerve preservation and low morbidity, comparable to those observed in resection of treatment-naive VS. In addition there were similar outcomes in terms of extent of resection between the two groups. The good outcomes reported here may reflect a confluence of factors which include patient selection, the experience of the attending surgeons, the use of more contemporary SRS dosing protocols during initial treatment for these patients, and divergent goals of therapy between cohorts

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.

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.

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: To validate a method of measuring angular depth of insertion (aDOI) as well as positional depth of each electrode contact in a cochlear implant by using intraoperative postinsertion skull radiographs. STUDY DESIGN: Retrospective review. SETTING: Tertiary referral center. PATIENTS: Intraoperative postinsertion radiographs obtained from 18 cochlear implant recipients were chosen for analysis. One high-resolution computer tomography scan of the head with the electrode in place was also analyzed. One cadaveric temporal bone with an inserted electrode provided additional data for analysis. INTERVENTION: aDOI and position of each electrode contact were measured from the radiographs using readily available software. High-resolution computer tomography imaging of the cochlea and electrode were reconstructed in three dimensions and used to simulate head rotation during intraoperative radiographs. The cadaveric temporal bone was imaged by x-ray at various acquisition angles. MAIN OUTCOME MEASURES: We evaluated the error introduced in measuring aDOI by assessing intra- and inter-rater variability. We also evaluated the error introduced by x-ray acquisition at nonstandardized angles by analyzing the three-dimensional construct and the cadaveric temporal bone. RESULTS: The concordance correlation coefficients for intrarater (0.991) and inter-rater (0.996) variability in aDOI measurement were excellent. The error introduced by nonstandardized x-ray acquisition angles was only -12.5 degrees to +15.8 degrees even at the limits of clinically relevant head rotation. CONCLUSIONS: The intraoperative postinsertion radiograph is sufficient for estimating positional depth of electrode contacts and the aDOI. This measure is robust in the face of nonstandardized x-ray acquisition angles, and shows good intra- and inter-rater variability.

OBJECTIVES: Commercially available cochlear implant systems attempt to deliver frequency information going down to a few hundred Hertz, but the electrode arrays are not designed to reach the most apical regions of the cochlea, which correspond to these low frequencies. This may cause a mismatch between the frequencies presented by a cochlear implant electrode array and the frequencies represented at the corresponding location in a normal-hearing cochlea. In the following study, the mismatch between the frequency presented at a given cochlear angle and the frequency expected by an acoustic hearing ear at the corresponding angle is examined for the cochlear implant systems that are most commonly used in the United States. DESIGN: The angular insertion of each of the electrodes on four different electrode arrays (MED-EL Standard, MED-EL Flex28, Advanced Bionics HiFocus 1J, and Cochlear Contour Advance) was estimated from X-ray. For the angular location of each electrode on each electrode array, the predicted spiral ganglion frequency was estimated. The predicted spiral ganglion frequency was compared with the center frequency provided by the corresponding electrode using the manufacturer's default frequency-to-electrode allocation. RESULTS: Differences across devices were observed for the place of stimulation for frequencies below 650 Hz. Longer electrode arrays (i.e., the MED-EL Standard and Flex28) demonstrated smaller deviations from the spiral ganglion map than the other electrode arrays. For insertion angles up to approximately 270 degrees , the frequencies presented at a given location were typically approximately an octave below what would be expected by a spiral ganglion frequency map, while the deviations were larger for angles deeper than 270 degrees . For frequencies above 650 Hz, the frequency to angle relationship was consistent across all four electrode models. CONCLUSIONS: A mismatch was observed between the predicted frequency and the default frequency provided by every electrode on all electrode arrays. The mismatch can be reduced by changing the default frequency allocations, inserting electrodes deeper into the cochlea, or allowing cochlear implant users to adapt to the mismatch. Further studies are required to fully assess the clinical significance of the frequency mismatch.