Hearing outcomes in children with Congenital Cytomegalovirus: A multi-center, single-enterprise experience.

BACKGROUND: Cytomegalovirus (CMV) is the most common cause of non-genetic sensorineural hearing loss (SNHL) in the United States; yet screening for congenital CMV (cCMV) remains controversial. CMV related SNHL can be present at birth, or develop in a delayed manner, and it is a consistent feature in children with either symptomatic or asymptomatic disease. A retrospective chart review was performed to determine the characteristics of patients diagnosed with cCMV and SNHL. METHODS: The electronic database warehouse of the Nemours Children's Health System (NCHS) was queried from 01/01/2004 to 10/05/2019. ICD 9 (771.1) and ICD 10 (B25.9, P35.1) diagnostic codes were used to identify patients throughout the system with a diagnosis of cCMV infection. Patient demographics including gender, race/ethnicity, age of diagnosis, results of newborn hearing screening (NBHS), detection and progression of hearing loss, presence of antiviral therapy, and frequency of monitoring were collected, and descriptive statistics performed. RESULTS: Of the 170 patients confirmed to have cCMV, 153 (90%) were symptomatic and 17 (10%) were asymptomatic. CNS involvement (63.5%), radiographic evidence of disease present (69.4%), and SNHL (50.6%) were the most common manifestations of the disease. Of these 170 patients, 83 (48.8%) were determined to have SNHL eligible for evaluation. For these patients with SNHL, the average time of hearing monitoring was 50.6 months. At the time of initial reported detection 63 of 83 (76%) had bilateral hearing loss and 20 (24%) had unilateral loss. Over the study period 3 (15%) progressed from unilateral to bilateral involvement, and 32 (47%) had a deterioration in hearing, with severe to profound SNHL in at least one ear identified at the last visit in 53 (64%) patients. Newborn hearing testing results were available for 69 (83%) of those with hearing loss and 26 patients passed initial testing. However, of the 26 patients who passed, 22 (85%) eventually developed SNHL by their last visit. Within our cohort, females with cCMV were significantly more likely to have SNHL than males with cCMV (62.3% versus 37.6%; p < 0.01). CONCLUSION: In the absence of targeted or universal cCMV screening, the majority of children identified with this condition present symptomatically. Approximately one half of children with symptomatic cCMV failed NBHS at birth while at least 25% develop SNHL later in life. Children with cCMV are at high risk of delayed onset loss and such children, particularly females, should be monitored closely.

Cochlear implantation in a patient with mucopolysaccharidosis IVA.

Mucopolysaccharidosis IVA (OMIM 253000; also known as Morquio A syndrome) is associated with skeletal, airway, and hearing abnormalities. Cochlear implantation is an effective intervention for patients with severe-to-profound hearing loss. Patients can gain substantial improvement in auditory performance, speech perception, and their quality of life from cochlear implantation. Although severe progressive sensorineural hearing loss is a common feature of mucopolysaccharidosis IVA, no detailed description of cochlear implantation for mucopolysaccharidosis IVA has been reported. To review the effectiveness and special considerations associated with cochlear implantation in patients with mucopolysaccharidosis IVA, we here report the case of cochlear implantation in mucopolysaccharidosis IVA by a multidisciplinary team. A retrospective chart review was conducted on a 34-year-old female with mucopolysaccharidosis IVA, who received a cochlear implant. Audiometric thresholds, speech perception scores, and cochlear implant processor mapping information were reviewed during the first 12 months following cochlear implantation. The results of audiological tests indicate improved hearing thresholds as well as remarkable enhancement of speech perception skills over 12 months of cochlear implant use. Cochlear implantation improved auditory performance in a mucopolysaccharidosis IVA patient with postlingually severe-to-profound sensorineural hearing loss. The benefits of cochlear implantation could be meaningful for other Morquio patients with progressive hearing loss, although the risks of surgery and anesthesia should be carefully considered by a multidisciplinary team of experts during the cochlear implant candidacy process.

Transmastoid access in branchio-oto-renal syndrome: A reappraisal of computed tomography imaging.

OBJECTIVE: To evaluate for temporal bone abnormalities that might affect transmastoid surgery such as cochlear implantation in cases of branchio-oto-renal syndrome (BOR). STUDY DESIGN: Retrospective review. METHODS: Qualitative assessment of temporal bone computed tomography imaging was performed by a neuroradiologist for 30 individuals with BOR (60 ears) and 20 controls with normal hearing (20 ears). Transmastoid access was assessed categorically across 4 features: tip development, cortex pneumatization, tegmen height, and facial recess pneumatization. The appearance of 4 standard landmarks (Koerner's septum, antrum, prominence of the horizontal semicircular canal, incudal short process) was also dichotomized as normal or abnormal. Data were compared using Fisher's exact testing. RESULTS: Mastoid height differed between the groups with tip underdevelopment noted in 72% of BOR ears vs. 40% of controls (p = 0.02), and a low tegmen was seen in 68% of BOR ears and 25% of controls (p < 0.01). Significant differences in pneumatization were also found for the mastoid cortex (28% non-pneumatized in BOR vs. 5% in controls; p = 0.03) and the facial recess (27% non-pneumatized in BOR vs. 0% in controls; p = 0.01). Standard landmarks were easily identified in all of the control mastoids. In the BOR group, Koerner's septum was abnormally located or absent in 45%, and the antrum was severely hypoplastic or absent in 50%. Similarly, the prominence of the horizontal semicircular canal and the short process of the incus were dysplastic in 73% (44/60) and 62% (37/60), respectively. CONCLUSIONS: Mastoid abnormalities are common in BOR syndrome. Restricted transmastoid access and abnormal or absent mastoid landmarks should be anticipated in those patients with BOR who become cochlear implant candidates. LEVEL OF EVIDENCE: 4.

Preliminary experience using a cochlear implant with a novel linear pedestal design.

OBJECTIVE: To assess the safety and efficiency of cochlear implantation using a novel device with a linear silastic pedestal (2 mm wide, 2 mm deep, 10 mm long) on the flat undersurface. METHODS: Operative times required to drill a linear groove (LG) for the new linear pedestal design were prospectively accrued for 46 implantations in 30 children (median age 3). Intra-operative safety was assessed during each case. Instances of dural exposure in the base of the LG were noted. Length of stay was also recorded as a secondary measure of efficiency. RESULTS: Across all surgeons, the mean time needed to create the LG was 1.9 ± 1.5 min (±SD) with a median time of 1.5 min (95% Cl: 1-2 min). The range in time was 1-10 min. No intraoperative complications occurred. Intended device positioning was confirmed with on-table post-operative x-rays in all cases. 43% of patients were discharged on the day of surgery. CONCLUSIONS: The novel linear pedestal design allows for deliberate device placement while adding little additional operative time and complexity, an improvement on our current standard of care.

Vestibular evoked myogenic potential testing as an objective measure of vestibular stimulation with cochlear implants.

OBJECTIVES/HYPOTHESIS: To determine if vestibular potentials could be elicited with electrical stimulation from cochlear implants. STUDY DESIGN: Prospective cohort study. METHODS: Vestibular responsiveness to electrical stimulation from cochlear implants was assessed via vestibular evoked myogenic potential (VEMP) testing in 53 pediatric and young adult patients. RESULTS: Thirty-one participants (58%) showed at least one vestibular potential in response to acoustic stimulation; 33 (62%) had an electrically evoked vestibular response. A cervical VEMP (cVEMP) was present in 45 of the 96 tested ears (47%) in response to acoustic stimulation, and in 34 ears (35%) with electrical stimulation. An ocular VEMP (oVEMP) was elicited acoustically in 25 ears (26%) and electrically in 34 (35%) ears. In the ears with absent responses to acoustic stimuli, electrically evoked cVEMPs and oVEMPs were present in 14 (27%) and 18 (25%) ears, respectively. Electric VEMPs demonstrated shorter latencies than acoustic VEMPs (P < .01). Whereas an increased prevalence of VEMPs was seen at high stimulation levels (P < .01), there was no difference between prevalence proportions with basal (electrode 3) or apical (electrode 20) stimulation (P > .05). CONCLUSIONS: VEMPs can be elicited with electrical stimulation in a proportion of children with cochlear implants, demonstrating current spread from the cochlea to the vestibular system. The presence of electric VEMPs in acoustically nonresponsive ears, along with the shorter latencies of electrically driven VEMPs, suggests that electrical current can bypass the otoliths and directly stimulate vestibular neural elements. LEVEL OF EVIDENCE: 4. Laryngoscope, 2016 127:E75-E81, 2017.

Assessment of Mastoid Function with Magnetic Resonance Imaging after Canal Wall Up Cholesteatoma Surgery.

OBJECTIVE: To use magnetic resonance imaging (MRI) to assess the extent of mastoid opacification after canal wall up (CWU) cholesteatoma surgery. MATERIALS AND METHODS: Thirty-five children in whom post-operative MRI had been obtained after CWU surgery. Cholesteatoma confined to the meso- and/or epi-tympanum was removed using a transcanal approach (n=18). More extensive disease required a combined approach tympanomastoidectomy (CAT, n=17). Mastoid opacification was assessed in both ears by a neuroradiologist blind to surgical details using an ordinal scale from 0 (no opacification) to 6 (completely opacified). The primary outcome measure was presence of normal mastoid ventilation, defined by evaluation of non-operative ears as a score ≤2. The presence of normal ventilation, as well as the raw opacification scores, were compared according to type of cholesteatoma surgery: 1) transcanal, with no mastoidectomy and 2) CAT. RESULTS: Mastoid ventilation was normal in 18 post-operative ears (51%). There was no significant difference in the proportion of normally ventilated mastoids in the CAT (n=17) and transcanal (n=18) groups (p=0.318; Fisher's exact). However, mastoid opacification scores were significantly higher in the CAT group than in the transcanal group (p=0.036; Mann-Whitney U). CONCLUSION: The mastoid frequently fails to become normally ventilated after cholesteatoma surgery. Subgroup analysis suggests cortical mastoidectomy does not increase the likelihood of normal mastoid ventilation after CWU cholesteatoma surgery. MRI provides a non-invasive tool to assess mastoid function, which contributes to the current debate on optimum surgical strategies for management of the mastoid in cholesteatoma surgery. Further research will determine whether this measure of mastoid health correlates with risk of recurrent cholesteatoma.

Stimulation from Cochlear Implant Electrodes Assists with Recovery from Asymmetric Perceptual Tilt: Evidence from the Subjective Visual Vertical Test.

Vestibular end organ impairment is highly prevalent in children who have sensorineural hearing loss (SNHL) rehabilitated with cochlear implants (CIs). As a result, spatial perception is likely to be impacted in this population. Of particular interest is the perception of visual vertical because it reflects a perceptual tilt in the roll axis and is sensitive to an imbalance in otolith function. The objectives of the present study were thus to identify abnormalities in perception of the vertical plane in children with SNHL and determine whether such abnormalities could be resolved with stimulation from the CI. Participants included 53 children (15.2 ± 4.0 years of age) with SNHL and vestibular loss, confirmed with vestibular evoked myogenic potential (VEMP) testing. Testing protocol was validated in a sample of nine young adults with normal hearing (28.8 ± 7.7 years). Perception of visual vertical was assessed using the static Subjective Visual Vertical (SVV) test performed with and without stimulation in the participants with cochleovestibular loss. Trains of electrical pulses were delivered by an electrode in the left and/or right ear. Asymmetric spatial orientation deficits were found in nearly half of the participants with CIs (24/53 [45%]). The abnormal perception in this cohort was exacerbated by visual tilts in the direction of their deficit. Electric pulse trains delivered using the CI shifted this abnormal perception towards center (i.e., normal; p = 0.007). Importantly, this benefit was realized regardless of which ear was stimulated. These results suggest a role for CI stimulation beyond the auditory system, in particular, for improving vestibular/balance function.

Advances in the diagnosis, management, and treatment of neonates with laryngeal disorders.

Neonatal airway obstruction due to laryngeal pathology may cause significant morbidity and even mortality. The most frequently occurring etiologies anatomically from superiorly to inferiorly include: laryngomalacia, saccular cyst, vocal fold paralysis, anterior glottic web, laryngeal atresia, laryngeal cleft, subglottic stenosis, and subglottic hemangioma. The pathophysiology, presentation, and treatment options for each of these entities are discussed with a focus on a multidisciplinary, evidence-based approach.

A preliminary report of percutaneous craniofacial osteoplasty in a rat calvarium.

OBJECTIVES/HYPOTHESIS: To evaluate the potential for injectable, permanent bone augmentation by assessing the biocompatability and bioactivity of subperiosteal hydroxylapatite (Radiesse) deposition in a rat model. STUDY DESIGN: Randomized controlled animal model. METHODS: Fourteen adult Sprague Dawley rats were injected in the parietal skull with 0.2 ml of hydroxylapatite (10 animals) or 0.2 ml of a carrier gel control (4 animals), using a subperiosteal injection technique on the right and a subcutaneous injection technique on the left. At 1, 3, and 6 months, three rats (1 negative control, 2 variables) were sacrificed and the calvaria were harvested. At 12 months, the remaining five rats were sacrificed. After each harvest, the specimens were processed and then examined under both light and polarized microscopy for new bone growth at the injection sites. RESULTS: The inflammatory response was limited with both hydroxylapatite and carrier injections. Injectables were still present 12 months after the injection. New bone formation was only observed when the injection was located deep to a disrupted periosteum The odds of new bone formation was 48.949 times higher (95% confidence intervals CI [2.637, 3759.961]; P=0.002) with subperiosteal hydroxylapatite injections compared to all other combinations of injection plane and injectable. CONCLUSIONS: This preliminary report of subperiosteal hydroxylapatite (Radiesse) injection in a rat model has verified the biocompatibility of injectable hydroxylapatite at the bony interface and suggests the potential for new bone formation. LEVEL OF EVIDENCE: N/A.

Resource analysis of tonsillectomy in children.

OBJECTIVES/HYPOTHESIS: To analyze variables that affect time and cost parameters of pediatric adenotonsillectomy. STUDY DESIGN: Longitudinal 7-month retrospective review of sequential tonsil and adenoid surgery at a university pediatric tertiary care hospital. METHODS: All children aged 2 to 12 years who underwent adenotonsillectomy from May 2008 to October 2008 had charts and billing records analyzed for variations in charges and times of adenotonsillectomy according to patient age, body mass index for age (BMIFA), American Society of Anesthesiologists (ASA) status, surgical indication, technology used, and teaching status of case. A total of 214 children had records reviewed. RESULTS: Statistically significant variations were observed for all measured parameters except for indications for surgery. Children 3 years and younger had shorter procedures (P = .005) and total operating room times (P = .037). Charges for supplies were lower for ASA 1 patients than for ASA 2 patients (P = .010). Obese children with elevated BMIFA required longer procedures (P = .039) and more expensive surgery (P = .003). Procedure times were shorter for Coblation (ArthroCare, Austin, TX) compared with electrocautery (P = .27) and for microdebrider compared with electrocautery (P < .001). Charges for Coblation were substantially higher (P < .001). Teaching cases took longer (P < .001). CONCLUSIONS: Charges and times for adenotonsillectomy surgery varied by patient age, BMIFA, ASA status, tonsillectomy technique, and teaching case status. Clinically salient differences were noted for ASA status, BMIFA, and surgical technique. This method of cost analysis provides useful information for resource management in tonsillectomy.

Incidence and management of rhinosinusitis after complex orbitofacial reconstruction.

OBJECTIVES/HYPOTHESIS: To examine the sinus-related sequelae of free flap reconstruction for complex orbitofacial defects. STUDY DESIGN: Retrospective chart review. METHODS: Demographic, clinical, and radiographic data on a series of 55 patients who had undergone free tissue transfer for orbitofacial reconstruction was retrospectively reviewed. Follow-up of ≥ 3 months was available for 49 patients. Outcome measures studied included clinical or radiographic evidence of sinusitis and the need for sinus surgery. RESULTS: The most commonly involved sinuses were the ethmoid (n = 40) and maxillary (n = 38) sinuses, and the anterolateral thigh was the most common flap used (n = 41). Clinical and/or radiographic sinusitis was evident in 21 patients (43%), and 10 patients (20%) required sinus surgery at some point during follow-up. Involvement of multiple sinuses in the initial orbitofacial surgery was associated with a significantly increased need for subsequent sinus surgery (P = 0.009). Adjuvant radiotherapy and adjuvant chemoradiotherapy were associated with a significantly increased risk for the development of rhinosinusitis (P = 0.045 and 0.016, respectively). CONCLUSION: Rhinosinusitis and the need for operative management of sinus obstruction are common in patients having undergone complex orbitofacial reconstruction. Careful management of the paranasal sinuses is an important component of the multidisciplinary treatment of such patients.

Use of the anterolateral thigh in cranio-orbitofacial reconstruction.

Objective. To detail the clinical outcomes of a series of patients having undergone free flap reconstruction of the orbit and periorbita and highlight the anterolateral thigh (ALT) as a workhorse for addressing defects in this region. Methods. A review of 47 patients who underwent free flap reconstruction for orbital or periorbital defects between September 2006 and May 2011 was performed. Data reviewed included demographics, defect characteristics, free flap used, additional reconstructive techniques employed, length of stay, complications, and follow-up. The ALT subset of the case series was the focus of the data reviewed for this paper. Selected cases were described to highlight some of the advantages of employing the ALT for cranio-orbitofacial reconstruction. Results. 51 free flaps in 47 patients were reviewed. 38 cases required orbital exenteration. The ALT was used in 33 patients. Complications included 1 hematoma, 2 wound infections, 3 CSF leaks, and 3 flap failures. Conclusions. Free tissue transfer allows for the safe and effective reconstruction of complex defects of the orbit and periorbital structures. Reconstructive choice is dependent upon the extent of soft tissue loss, midfacial bone loss, and skullbase involvement. The ALT provides a versatile option to reconstruct the many cranio-orbitofacial defects encountered.