The impact of cochlear implantation on speech and language outcomes in children with asymmetric sensorineural hearing loss.

INTRODUCTION: Cochlear implantation (CI) is considered in children with asymmetric sensorineural hearing loss (ASHL), or severe to profound sensorineural hearing loss in one ear, and better hearing contralaterally. The benefits of a CI in these children are not well established. This study investigated the impact of unilateral CI on speech and language outcomes in pediatric patients with ASHL. METHODS: The retrospective chart review identified pediatric patients who underwent cochlear implantation for ASHL at a tertiary single center institution between 2014 and 2017. Children who underwent cochlear implantation in the poorer ear and maintained a hearing aid in the better ear were included, while children with cognitive impairment or prior cochlear nerve deficiency and auditory neuropathy were excluded. Speech and language measures were evaluated prior to implantation and at regular six-month intervals postoperatively. Ear specific Audiometry was evaluated at every visit and varied developmentally appropriate speech recognition tasks, including questionnaires, closed-set, and open-set measures, accounted for advancing childhood milestones. Specific measures of speech articulation, expressive and receptive language, and vocabulary were assessed including: Goldman Fristoe Test of Articulation (GFTA-3), Clinical Evaluation of Language Fundamentals (CELF-P2 or CELF-5), Receptive One-Word Picture Vocabulary Test (ROWPVT) and Expressive One Word Picture Vocabulary Test (EOWPVT). The mean difference and standard deviation of speech and language outcomes at preoperative and postoperative evaluations were calculated. The change in outcomes were further assessed over each post-operative time point. RESULTS: Twenty-six children with asymmetric sensorineural hearing loss were included in the analysis, with 11 (42%) having bilateral enlarged vestibular aqueducts (EVA). At time of CI, unaided mean Pure Tone Average (PTA-4) for the better ear was 62Db HL, and worse ear was 92Db HL. There was an improvement in speech recognition in the implanted ear as expected. All preoperative speech and language evaluations were conducted within 7 months of cochlear implantation. Twenty-three patients had at least 1 postoperative re-evaluation. Fourteen children had preoperative and postoperative assessments with the same tool, with 13 patients (93%) showing improvement in at least 1 domain. For patients with repeated assessment within 12 months of surgery, we observed an average improvement in standard scores of: GFTA-3 (+11.2), CELF-P2 Core Language (+10.3), CELF-5 Core Language (+7.0), ROWPVT (+2.8) and EOWPVT (+4.5). Individual results are described. CONCLUSION: Improvement in speech and language measures can be demonstrated in children undergoing unilateral cochlear implantation for asymmetric sensorineural hearing losses. These children, who are not typical CI candidates, can benefit from a CI in the poorer ear.

Early pediatric Cochlear implantation: An update.

The criteria and candidacy for pediatric cochlear implantation (CI) has significantly transformed over the past few decades and continues to evolve with technological advancements, and recognition of benefit in more diverse populations. Prolonged auditory deprivation among patients with profound sensorineural hearing loss has been shown to cause widespread degeneration in the central auditory system. Thus, there is increasing evidence advocating for earlier implantation within a critical neuroplastic window. However, there is a lack of consensus on this optimal age of implantation. Historically, there were concerns regarding surgical feasibility and safety, anesthesia risk, and logistical considerations in very young infants <12 months. Recent literature has investigated surgical safety and anesthesia risk as well speech and language outcomes with early implantation, resulting in the long-awaited reduction in approved age by the FDA (<9 months for certain devices). This article reviews logistical considerations, surgical safety, anesthesia risk, and language developmental outcomes associated with early CI (<12 months).

Electrocochleography Observations in a Series of Cochlear Implant Electrode Tip Fold-Overs.

OBJECTIVE: Tip fold-over is a rare but serious complication of cochlear implant (CI) surgery. The purpose of this study was to present intraoperative electrocochleography (ECochG) observations in a series of CI electrode tip fold-overs. PATIENTS: Five pediatric subjects undergoing CI surgery through a round window (RW) approach with a perimodiolar electrode array, who were diagnosed with either auditory neuropathy spectrum disorder or enlarged vestibular aqueduct. INTERVENTIONS: Intraoperative RW ECochG during CI surgery: tone burst stimuli were presented from 95 to 110 dB SPL. MAIN OUTCOME MEASURES: Magnitude and phase characteristics of ECochG responses obtained intraoperatively before and immediately after electrode insertion were examined for patients with and without tip fold-over. RESULTS: Three subjects presented with tip fold-over and two formed the control group. Among fold-over cases, one participant exhibited an inversion in the starting phase of the cochlear microphonic response and a decrease in spectral magnitude from pre- to postinsertion. Both subjects who did not exhibit a change in phase had an increase in the ECochG-total response (ECochG-TR) magnitude. No case in the control group exhibited a change in starting phase. In regard to the ECochG-TR, all controls showed a decrease in the magnitude. CONCLUSIONS: Despite the small number of patients, heterogeneous ECochG response patterns were observed within the fold-over group. Though these results are not conclusive, they can serve as a framework to begin to understand ECochG's utility in detecting intraoperative tip fold-over.

Timing of newborn hearing screening in the neonatal intensive care unit: implications for targeted screening for congenital cytomegalovirus infection.

OBJECTIVE: To determine when infants in the neonatal intensive care unit (NICU) have the first hearing screen performed, and thus inform targeted testing for cytomegalovirus (CMV)-related hearing loss. STUDY DESIGN: Retrospective review of electronic health records of infants admitted to a Level 4 outborn NICU and had a first hearing screen performed from 8/2016-8/2018. RESULT: Among 1498 infants, 546 (36%) had a first hearing screen performed at age >21 days when a positive CMV PCR test cannot distinguish congenital from postnatal CMV acquisition. While most infants tested at >21 days of age were <34 weeks' gestational age (71%), 18% (n = 100) and 11% (n = 59) were ≥34 and ≥37 weeks' gestation, respectively. CONCLUSION: Targeted CMV testing for failed hearing screen in the NICU is problematic as 36% of infants did not have a hearing screen performed before 21 days of age, supporting the need for CMV screening at NICU admission.

The otolaryngologist's and anesthesiologist's collaborative role in a pandemic: A large quaternary pediatric center's experience with COVID-19 preparation and simulation.

There has been a rapid global spread of a novel coronavirus, the Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2), which originated in Wuhan China in late 2019. A serious threat of nosocomial spread exists and as such, there is a critical necessity for well-planned and rehearsed processes during the care of the COVID-19 positive and suspected patient to minimize transmission and risk to healthcare providers and other patients. Because of the aerosolization inherent in airway management, the pediatric otolaryngologist and anesthesiologist should be intimately familiar with strategies to mitigate the high-risk periods of viral contamination that are posed to the environment and healthcare personnel during tracheal intubation and extubation procedures. Since both the pediatric otolaryngologist and anesthesiologist are directly involved in emergency airway interventions, both specialties impact the safety of caring for COVID-19 patients and are a part of overall hospital pandemic preparedness. We describe our institutional approach to COVID-19 perioperative pandemic planning at a large quaternary pediatric hospital including operating room management and remote airway management. We outline our processes for the safe and effective care of these patients with emphasis on simulation and pathways necessary to protect healthcare workers and other personnel from exposure while still providing safe, effective, and rapid care.

Pediatric lateral graft tympanoplasty A review of 78 cases.

OBJECTIVES: The optimal timing and technique for repairing chronic tympanic membrane (TM) perforations in pediatric patients remains controversial. The objectives are to determine the surgical and hearing outcomes of pediatric lateral graft tympanoplasty at a tertiary teaching hospital. METHODS: A retrospective review was conducted for pediatric lateral graft tympanoplasties performed for chronic TM perforations by a single surgeon over a four-year period. Primary and secondary outcomes were graft failure rate and hearing outcomes, respectively. RESULTS: 78 cases were analyzed. The mean age at time of surgery was 10.3 years (range 5-18 years). Mean follow-up was 11.0 months; 27 patients had follow-up >1 yr. Most patients were non-syndromic (85.9%), had a history of bilateral Eustachian tube dysfunction (ETD) (59%) and presented with marked myringosclerosis (73.1%). Thirty-three percent of cases were revision tympanoplasties. A learner surgeon (resident or fellow) was present in 89.7% of cases. Successful closure of the TM was achieved in 97.4% (76/78) of cases and 92.6% (25/27) of cases with >1-year follow-up. No obvious difference in graft failure was noted with regards to age at time of surgery, perforation size, history of bilateral ETD, presence of a learner surgeon, myringosclerosis, presence of syndromic features, or history of prior tympanoplasty. Ninety-one percent of patients either improved hearing or preserved their conductive hearing deficit. Poorer hearing outcomes were only associated with post-operative blunting. CONCLUSIONS: Pediatric lateral graft tympanoplasty is effective in repairing chronic perforations with excellent hearing outcomes. Common quoted predictors of surgical outcome such as age at the time of surgery, syndromic features, history of previous myringoplasty, perforation size, and ETD dysfunction were not associated with graft failure in our series.

Audiometric findings in children with unilateral enlarged vestibular aqueduct.

OBJECTIVE: To evaluate the prevalence of bilateral hearing loss in children with unilateral enlarged vestibular aqueduct (EVA) at a single institution. METHODS: A retrospective case review was performed at a tertiary care pediatric referral center involving children with radiologic findings of unilateral EVA and normal labyrinthine anatomy of the contralateral ear diagnosed via CT and/or MRI. The main outcome measure of interest is the number of patients with unilateral EVA who were diagnosed with bilateral hearing loss. RESULTS: Sixty-one pediatric patients were identified. The mean audiometric follow-up was 48.2 months (0-150). Three (4.9%) patients with unilateral EVA were noted to have bilateral hearing loss, and this rate was not significantly different (p = 1.0) from the rate reported in a comparison group of patients with contralateral hearing loss (6.0%) without an EVA. The pure-tone average (defined as the average dB HL at 500, 1000, 2000, and 4000 Hz) in the group with bilateral hearing loss was 31.3 dB HL in the better hearing ear and 79.6 dB HL in the worse hearing ear, with the difference being statistically significant (p = 0.02). In the unilateral EVA patients without contralateral hearing loss (n = 56, 91.8%), the PTA was 9.4 dB HL in the better hearing ear and 51.9 dB HL in the worse hearing ear, with the difference being statistically significant (p < 0.001). Two patients (3.3%) with unilateral EVA were found to have hearing within normal limits bilaterally. The EVA was ipsilateral to the worse hearing ear in all cases. CONCLUSION: The prevalence of bilateral hearing loss in children with unilateral EVA appears to be low. Specifically, it may be no different than the rate of contralateral hearing loss in children with unilateral hearing loss without an EVA. The present report is somewhat different than the previously described prevalence in the literature. This difference could be related to the imaging type and diagnostic criteria used, the patients included, the source of the identified patents, and the overall population of patients studied.

Parent perspectives on multidisciplinary pediatric hearing healthcare.

INTRODUCTION: Family-centered healthcare demands that families provide input regarding the care of their children. Very little is known, however, about how families perceive their experience in different types of multidisciplinary team models, and specifically, in the multidisciplinary setting currently utilized in many pediatric hearing clinics. METHODS: Quantitative and qualitative parent survey responses were collected and analyzed in a tertiary care pediatric medical center after a one-day multidisciplinary assessment clinical appointment. Questions pertained to information across five domains, including overall experience, diagnosis, treatment plan formulation, additional testing, and resources. Quantitative responses were analyzed descriptively while qualitative responses were evaluated using content analysis to derive themes. Quantitative and qualitative data were evaluated separately and then compared to delineate themes for strengths and weaknesses. RESULTS: Overall, high satisfaction was evident in both quantitative and qualitative responses. Results suggested that a one-day multidisciplinary assessment appointment may contribute to parents feeling overwhelmed by information shared and not fully understanding which disciplines are providing care. Analysis revealed a specific area of weakness in our particular setting was inadequate provision of information about functional hearing (e.g., listening socially and academically). Results contributed to a change from a multidisciplinary team model to an interdisciplinary care coordination approach to pediatric hearing healthcare. CONCLUSIONS: Understanding parent perspectives and expectations is the corner stone of family-centered care and may ultimately influence a child's developmental outcome. A systematic way of evaluating parent perspectives on the clinical process can influence service delivery and help children with hearing loss meet their potential.

The use of botulinum toxin for pediatric cricopharyngeal achalasia.

OBJECTIVES: Cricopharyngeal achalasia is an uncommon cause of feeding difficulties in the pediatric population, and is especially rare in infants. Traditional management options include dilation or open cricopharyngeal myotomy. The use of botulinum toxin has been preliminarily reported for cricopharyngeal achalasia in children as a modality for diagnosis and management. This study describes the use of botulinum toxin as a definitive treatment for pediatric cricopharyngeal achalasia. METHODS: A retrospective analysis was performed of three patients who were diagnosed with cricopharyngeal achalasia and underwent botulinum toxin injections to the cricopharyngeus muscle. The charts were reviewed for etiology, botulinum toxin dosage delivered, length of follow-up, postoperative need for nasogastric tube placement, and swallow studies. RESULTS: A total of 7 botulinum toxin injections into the cricopharyngeus muscle were performed in three infants with primary cricopharyngeal achalasia between April 2006 and February 2011. Mean dosage was 23.4 units per session (range: 10-44 units), or 3.1 U/kg (range: 1.4-5.3 U/kg). Mean interval period between injections was 3.3 months (range: 2.7-4.0 months). Mean follow-up period was 22.1 months (range: 3.4-44.5 months). One patient required hospital readmission after injection for presumed aspiration but recovered without need for surgical intervention. No long-term complications were noted post-operatively. All patients improved clinically and ultimately had their nasogastric feeding tubes removed. CONCLUSIONS: Botulinum toxin appears to be a safe and effective option in the management of primary cricopharyngeal achalasia in children, and may prevent the need for myotomy.

The use of botulinum toxin for pediatric cricopharyngeal achalasia.

OBJECTIVES: Cricopharyngeal achalasia is an uncommon cause of feeding difficulties in the pediatric population, and is especially rare in infants. Traditional management options include dilation or open cricopharyngeal myotomy. The use of botulinum toxin has been preliminarily reported for cricopharyngeal achalasia in children as a modality for diagnosis and management. This study describes the use of botulinum toxin as a definitive treatment for pediatric cricopharyngeal achalasia. METHODS: A retrospective analysis was performed of three patients who were diagnosed with cricopharyngeal achalasia and underwent botulinum toxin injections to the cricopharyngeus muscle. The charts were reviewed for etiology, botulinum toxin dosage delivered, length of follow-up, post-operative need for nasogastric tube placement, and swallow studies. RESULTS: A total of 7 botulinum toxin injections into the cricopharyngeus muscle were performed in three infants with primary cricopharyngeal achalasia between April 2006 and February 2011. Mean dosage was 23.4 units per session (range: 10-44 units), or 3.1 U/kg (range: 1.4-5.3 U/kg). Mean interval period between injections was 3.3 months (range: 2.7-4.0 months). Mean follow-up period was 22.1 months (range: 3.4-44.5 months). One patient required hospital readmission after injection for presumed aspiration but recovered without need for surgical intervention. No long-term complications were noted post-operatively. All patients improved clinically and ultimately had their nasogastric feeding tubes removed. CONCLUSIONS: Botulinum toxin appears to be a safe and effective option in the management of primary cricopharyngeal achalasia in children, and may prevent the need for myotomy.

Clinical, radiographic, and audiometric predictors in conservative management of vestibular schwannoma.

OBJECTIVE: Vestibular schwannomas (VS) can be managed by observation. The goals were to examine clinical, radiographic, and audiometric variables at presentation and during observation that may predict which patients fail conservative management. METHODS: A retrospective chart review was performed of 202 patients who elected observation primarily. Data collection included presenting symptoms, symptom progression, tumor size, audiologic measures, and global clinical outcomes. Univariate and multivariate analyses were performed. RESULTS: Follow-up ranged from 1 month to 16 years (mean, 2.48 yr). Nineteen patients (9.4%) in the study group failed. Disequilibrium as a presenting symptom appeared more often in patients who failed observation (58% versus 32%; p = 0.039), as did new-onset disequilibrium. Presenting tumor size differed for patients who failed conservative management, with a mean of 14.0 versus 8.4 mm (p = 0.0006). Neurotologic complications compared favorably to those treated with primary surgery or radiotherapy. CONCLUSION: Patients with subjective disequilibrium at presentation and subjective disequilibrium developed during observation may be more likely to fail conservative management. Increased tumor size at presentation also may indicate the same, although no threshold could be achieved.

Aesthetic considerations in mandibular reconstruction.

As free tissue transfer methods have improved, vascular bone grafting has become state of the art for reconstruction of mandibular defects. Prior studies have focused on flap survival and functional outcomes. The reconstructive surgeon should also strive to attain lofty aesthetic goals for this group of patients. The best results are achieved when patient factors, flap selection, treatment planning, and surgical techniques are all considered and properly selected.