Is "no response" on diagnostic auditory brainstem response testing an indication for cochlear implantation in children?

OBJECTIVES: To compare the results of a "no response" (NR) result on auditory brainstem response (ABR) testing with those of behavioral pure-tone audiometry and ultimate clinical tracking to cochlear implantation (CI). DESIGN: Retrospective review of pediatric patients who underwent multifrequency ABR testing in a 5 year span. Total of 1143 pediatric patients underwent ABR testing during the study period and 105 (9.2%) were identified with bilateral NR based on absent responses to both click and tone burst stimuli. For the children with NR, various clinical parameters were evaluated as these children progressed through the CI evaluation process. Children were grouped based on whether they underwent ABRs for diagnostic or for confirmatory purposes. RESULTS: Of the 105 children who met inclusion criteria, 94 had sufficient follow-up to be included in this analysis. Ninety-one (96.8%) of 94 children with bilateral NR ABRs were ultimately recommended for and received a CI. Three (3.2%) children were not recommended for implantation based on the presence of multiple comorbidities rather than auditory factors. None of the children (0%) had enough usable residual hearing to preclude CI. For those who had diagnostic ABRs, the average time at ABR testing was 5.4 months (SD 6.2, range 1-36) and the average time from ABR to CI was 10.78 months (SD 5.0, range 3-38). CONCLUSIONS: CI should tentatively be recommended for children with a bilateral NR result with multifrequency ABR, assuming confirmatory results with behavioral audiometric testing. Amplification trials, counseling, and auditory-based intervention therapy should commence but not delay surgical intervention, as it does not appear to change the eventual clinical course. Children not appropriate for this "fast-tracking" to implantation might include those with significant comorbidities, auditory neuropathy spectrum disorder, and unreliable or poorly correlated results on behavioral audiometric testing.

Post-operative budesonide irrigations for patients with polyposis: a blinded, randomized controlled trial.

OBJECTIVE: To compare normal saline (NS) vs. NS+budesonide irrigations in post- functional endoscopic sinus surgery (FESS) patients with chronic rhinosinusitis with polyposis (CRSwNP). Currently, no evidence exists for NS+budesonide irrigation over NS irrigation alone. STUDY DESIGN: Prospective, single-blind, randomized controlled trial. METHODS: Subjects were prospectively enrolled to NS or NS+budesonide arms. Patients were evaluated at pre-operative and three post-operative visits (POV): POV1 (1-2 weeks post-op), POV2 (3-8 weeks post-op), and POV3 (3-6 months post-op). Patients were evaluated by three quality of life (QOL) questionnaires (SNOT-22, RSOM-31, and RSDI) and two olfaction scores (UPSIT and the PEA test). RESULTS: Fifty patients were randomized, with 25 patients in the NS arm and 25 patients in the NS+budesonide arm. Two patients had unexpected pathology and were excluded from the study. By POV2 and POV3, patients experienced a significant improvement in all three QOL surveys, although the degree of improvement between arms was not significant up through POV3. Neither arm experienced significant olfactory improvement up through POV3. CONCLUSIONS: While both NS and NS+budesonide treatments improve QOL for post-FESS patients, neither intervention significantly increases QOL as compared to the other. Olfaction was not significantly improved in either treatment group.

Pedicled extranasal flaps in skull base reconstruction.

Cerebrospinal fluid (CSF) leaks most commonly arise during or after skull base surgery, although they occasionally present spontaneously. Recent advances in the repair of CSF leaks have enabled endoscopic endonasal surgery to become the preferred option for management of skull base pathology. Small defects (<1 cm) can be repaired by multilayered free grafts. For large defects (>3 cm), pedicled vascular flaps are the repair method of choice, resulting in much lower rates of postoperative CSF leaks. The pedicled nasoseptal flap (NSF) constitutes the primary reconstructive option for the vast majority of skull base defects. It has a large area of potential coverage and high rates of success. However, preoperative planning is required to avoid sacrificing the NSF during resection. In cases where the NSF is unavailable, often due to tumor involvement of the septum or previous resection removing or compromising the flap, other flaps may be considered. These flaps include intranasal options - inferior turbinate or middle turbinate flaps - as well as regional pedicled flaps: pericranial flap, temporoparietal fascial flap, or palatal flap. More recently, novel alternatives such as the pedicled facial buccinator flap and the pedicled occipital galeopericranial flap have been added to the arsenal of options for skull base reconstruction. Characteristics of and appropriate uses for each flap are described.

Cochlear implantation in unique pediatric populations.

PURPOSE OF REVIEW: Over the last decade, the selection criteria for cochlear implantation have expanded to include children with special auditory, otologic, and medical problems. Included within this expanded group of candidates are those children with auditory neuropathy spectrum disorder, cochleovestibular malformations, cochlear nerve deficiency, associated syndromes, as well as multiple medical and developmental disorders. Definitive indications for cochlear implantation in these unique pediatric populations are in evolution. This review will provide an overview of managing and habilitating hearing loss within these populations with specific focus on cochlear implantation as a treatment option. RECENT FINDINGS: Cochlear implants have been successfully implanted in children within unique populations with variable results. Evaluation for cochlear implant candidacy includes the core components of a full medical, audiologic, and speech and language evaluations. When considering candidacy in these children, additional aspects to consider include disorder-specific surgical considerations and child/caregiver counseling regarding reasonable postimplantation outcome expectations. SUMMARY: Cochlear implants are accepted as the standard of care for improving hearing and speech development in children with severe-to-profound hearing loss. However, children with sensorineural hearing loss who meet established audiologic criteria for cochlear implantation may have unique audiologic, medical, and anatomic characteristics that necessitate special consideration regarding cochlear implantation candidacy and outcome. Individualized preoperative candidacy and counseling, surgical evaluation, and reasonable postoperative outcome expectations should be taken into account in the management of these children.

Cochlear implant-evoked cortical activation in children with cochlear nerve deficiency.

OBJECTIVE: To report the results of cochlear implant-elicited cortical auditory evoked potentials (eCAEP) in children with cochlear nerve deficiency (CND). STUDY DESIGN: Case control series. SETTING: Tertiary academic referral center. PATIENTS: Seven children with CND that have a cochlear implant in their affected ear. Four children without CND served as controls. INTERVENTION(S): eCAEPs were elicited by activation of individual cochlear implant electrodes. MAIN OUTCOME MEASURE(S): Onset responses (P1-N1-P2 complex). RESULTS: Three of 7 CND children demonstrated eCAEP responses across a broad range of electrodes despite having limited or no open set speech perception abilities using their implants. Two of these children had eCAEPs that were characterized by substantial variability in latency, amplitude, and number of electrodes with identifiable responses. The remaining 4 ears with CND and poor speech perception had multiphasic responses that are inconsistent with eCAEPs. Non-CND ears with excellent speech perception abilities demonstrated robust responses on all electrodes stimulated. CONCLUSION: Abent eCAEP responses were indicative of poor open-set speech perception skills in all cases. However, eCAEP onset responses were measurable in some children with imaging evidence of CND, indicating probable cochlear nerve hypoplasia rather than aplasia. That some children with CND and poor speech perception had robust eCAEPs in some instances makes this particular measure of limited use for predicting good speech perception outcomes after cochlear implantation in these children. The origin of multiphasic responses remains to be determined but may be of somatosensory origin in some instances.