Early-stage use of hearing aids preserves auditory cortical structure in children with sensorineural hearing loss.

Hearing is critical to spoken language, cognitive, and social development. Little is known about how early auditory experiences impact the brain structure of children with bilateral sensorineural hearing loss. This study examined the influence of hearing aid use and residual hearing on the auditory cortex of children with severe to profound congenital sensorineural hearing loss. We evaluated cortical preservation in 103 young pediatric cochlear implant candidates (55 females and 48 males) by comparing their multivoxel pattern similarity of auditory cortical structure with that of 78 age-matched children with typical hearing. The results demonstrated that early-stage hearing aid use preserved the auditory cortex of children with bilateral congenital sensorineural hearing loss. Children with less residual hearing experienced a more pronounced advantage from hearing aid use. However, this beneficial effect gradually diminished after 17 months of hearing aid use. These findings support timely fitting of hearing aids in conjunction with early implantation to take advantage of neural preservation to maximize auditory and spoken language development.

The Extent of Hearing Input Affects the Plasticity of the Auditory Cortex in Children With Hearing Loss: A Preliminary Study.

PURPOSE: This study investigated to what extent residual hearing and rehabilitation options (e.g., hearing aids [HAs]) affect the auditory cortex in children with hearing loss. METHOD: Twenty-one children with bilateral congenital sensorineural hearing loss who were candidates for cochlear implantation were recruited. Voxel-based morphometry analysis was conducted to assess the gray matter (GM) volume in the auditory cortex. Children's residual hearing was measured by pure-tone audiometry at different frequencies. Multiple linear regression models were conducted to examine the effects of residual hearing and the use of HAs on GM volume in the auditory cortex with the control of age and gender. RESULTS: Children with more residual hearing at high frequencies had larger GM volume ratio (corrected by total intracranial volume) in the left Heschl's gyrus (r = -.545, p = .013). An interaction effect between residual hearing and the use of HAs suggested that the effect of residual hearing on GM ratio was moderated by the use of HAs (ß = -.791, p = .020). Compared with children with less residual hearing, children who had more residual hearing benefited more from longer use of HAs in terms of a larger GM ratio. CONCLUSIONS: Our preliminary findings highlight the impact of residual hearing on the neuroanatomy of the auditory cortex in children with hearing loss. Moreover, our results call for more auditory input via HAs for children with more residual hearing to preserve the auditory cortex before cochlear implantation. For children with less residual hearing who might receive limited benefit from HAs, an early cochlear implant would be necessary.

Harnessing the Power of Artificial Intelligence in Otolaryngology and the Communication Sciences.

Use of artificial intelligence (AI) is a burgeoning field in otolaryngology and the communication sciences. A virtual symposium on the topic was convened from Duke University on October 26, 2020, and was attended by more than 170 participants worldwide. This review presents summaries of all but one of the talks presented during the symposium; recordings of all the talks, along with the discussions for the talks, are available at https://www.youtube.com/watch?v=ktfewrXvEFg and https://www.youtube.com/watch?v=-gQ5qX2v3rg . Each of the summaries is about 2500 words in length and each summary includes two figures. This level of detail far exceeds the brief summaries presented in traditional reviews and thus provides a more-informed glimpse into the power and diversity of current AI applications in otolaryngology and the communication sciences and how to harness that power for future applications.

American Cochlear Implant Alliance Task Force Guidelines for Clinical Assessment and Management of Cochlear Implantation in Children With Single-Sided Deafness.

More children with single-sided deafness (SSD) are receiving cochlear implants (CIs) due to the expansion of CI indications. This unique group of pediatric patients has different needs than the typical recipient with bilateral deafness and requires special consideration and care. The goal of cochlear implantation in these children is to provide bilateral input to encourage the development of binaural hearing. Considerations for candidacy and follow-up care should reflect and measure these goals. The purpose of this document is to review the current evidence and provide guidance for CI candidacy, evaluation, and management in children with SSD.

Gorham Stout disease of the temporal bone with cerebrospinal fluid leak.

Gorham Stout disease (GSD) is a rare disease characterized by the proliferation of endothelial lined vessels and replacement of bone by fibrous tissue. The main imaging features are progressive osteolysis and cortical resorption. Temporal bone involvement is rare but presents as a destructive bone lesion that may be misinterpreted as more common lytic processes in the pediatric population, such as infection or Langerhans cell histiocytosis. GSD of the temporal bone is associated with cerebrospinal fluid (CSF) leaks, may present with otorrhea, and can mimic other causes of ear drainage. Here, we report the clinical course, imaging features, and outcomes of a 3-year-old girl with GSD of the temporal bone presenting with CSF leak initially attributed to infection.

Cochlear Implantation Can Improve Auditory Skills, Language and Social Engagement of Children With Autism Spectrum Disorder.

OBJECTIVE: To review outcomes of cochlear implantation (CI) in children diagnosed with autism spectrum disorder (ASD). STUDY DESIGN: Retrospective case review and parent survey. SETTING: Tertiary care children's hospital. PATIENTS: Thirty children with ASD who underwent CI between 1991 and 2018. Mean age at CI = 3.5 years (0.8-11.8), mean age at diagnosis of ASD = 5.1 years (2.0-15.0) (22/30 diagnosed after CI), mean follow-up = 10.5 years (1.4-21.6). Parents of 7 children returned a survey. INTERVENTION: Unilateral or bilateral cochlear implantation. MAIN OUTCOME MEASURES: Speech perception; expressive communication mode; educational placement; social engagement; consistency of CI use; parent survey of child behavior change. RESULTS: Thirty-three percent of all and 45% of the 22 consistent device users developed measurable open-set speech perception by an average of 4.5 years of device use. Educational placement at last follow-up included 13% mainstreamed without interpreter, 50% Special Education programs, 10% therapeutic residential or day programs, 23% total communication programs, and one home schooled. Spoken language alone was used by 31% and spoken plus sign by 14%, with the remainder using sign alone, augmentative communication devices or no mode of communication. By parent report, 86% showed improvement in social engagement compared to pre-CI. Survey results showed the behaviors most frequently ranked as most affected by CI were communication and attention, while awareness of environment had the lowest (most affected) mean ranking. CONCLUSIONS: Findings support a growing body of literature that cochlear implantation has the potential to improve auditory skills, language, and enhance social engagement in some deaf children with autism spectrum disorder.

Impact of Cochlear Implant With Diametric Magnet on Imaging Access, Safety, and Clinical Care.

OBJECTIVES/HYPOTHESIS: Review safety and effectiveness of magnetic resonance imaging (MRI) of patients implanted with a cochlear implant (CI) containing a diametric magnet housed within the undersurface of the device. STUDY DESIGN: Retrospective chart review with additional review of MRI at a tertiary-care children's hospital. METHODS: Seven patients with mean age of 8.4 years (range = 1.3-19 years) with a diametric magnet in situ during MRI. The intervention comprised one or more sessions of 1.5 T or 3.0 T MRI without a head wrap. The main outcome measures were the occurrence of magnet-related complications including discomfort and magnet displacement, use of anesthesia or sedation, and clinical usefulness of MRI studies. RESULTS: Seven CI recipients underwent 17 episodes of 1.5 or 3.0 T MRI with an in situ diametric magnet. Thirteen of 17 (76%) MRI sessions were completed in awake patients. No patients had device-related discomfort. No magnet-related complications occurred. Thirteen of 14 (93%) brain studies were clinically useful despite artifacts. CONCLUSIONS: The diametric magnet enabled MRI with magnet in situ without the discomfort or magnet displacement associated with removable axial magnets. The reduction in MRI magnet-related complications occurred because torque is not directed perpendicular and outward from the plane of the magnet, and the magnet is securely contained within its housing. The design of this device increased access and reduced the need for sedation or anesthesia. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:E952-E956, 2021.

Safety and Effectiveness of Cochlear Implantation of Young Children, Including Those With Complicating Conditions.

OBJECTIVE: Determine safety and effectiveness of cochlear implantation of children under age 37 months, including below age 12 months. STUDY DESIGN: Retrospective review. SETTING: Tertiary care children's medical center. PATIENTS: 219 children implanted before age 37 mos; 39 implanted below age 12 mos and 180 ages 12-36 mos. Mean age CI = 20.9 mos overall; 9.4 mos (5.9-11.8) and 23.4 mos (12.1-36.8) for the two age groups, respectively. All but two ≤12 mos (94.9%) received bilateral implants as did 70.5% of older group. Mean follow-up = 5.8 yrs; age last follow-up = 7.5 yrs, with no difference between groups. INTERVENTIONS: Cochlear implantation. MAIN OUTCOME MEASURES: Surgical and anesthesia complications, measurable open-set speech discrimination, primary communication mode(s). RESULTS: Few surgical complications occurred, with no difference by age group. No major anesthetic morbidity occurred, with no critical events requiring intervention in the younger group while 4 older children experienced desaturations or bradycardia/hypotension. Children implanted under 12 mos developed open-set earlier (3.3 yrs vs 4.3 yrs, p ≤ 0.001) and were more likely to develop oral-only communication (88.2% vs 48.8%, p ≤ 0.001). A significant decline in rate of oral-only communication was present if implanted over 24 months, especially when comparing children with and without additional conditions associated with language delay (8.3% and 35%, respectively). CONCLUSIONS: Implantation of children under 37 months of age can be done safely, including those below age 12 mos. Implantation below 12 mos is positively associated with earlier open-set ability and oral-only communication. Children implanted after age 24 months were much less likely to use oral communication exclusively, especially those with complex medical history or additional conditions associated with language delay.

Neural preservation underlies speech improvement from auditory deprivation in young cochlear implant recipients.

Although cochlear implantation enables some children to attain age-appropriate speech and language development, communicative delays persist in others, and outcomes are quite variable and difficult to predict, even for children implanted early in life. To understand the neurobiological basis of this variability, we used presurgical neural morphological data obtained from MRI of individual pediatric cochlear implant (CI) candidates implanted younger than 3.5 years to predict variability of their speech-perception improvement after surgery. We first compared neuroanatomical density and spatial pattern similarity of CI candidates to that of age-matched children with normal hearing, which allowed us to detail neuroanatomical networks that were either affected or unaffected by auditory deprivation. This information enables us to build machine-learning models to predict the individual children's speech development following CI. We found that regions of the brain that were unaffected by auditory deprivation, in particular the auditory association and cognitive brain regions, produced the highest accuracy, specificity, and sensitivity in patient classification and the most precise prediction results. These findings suggest that brain areas unaffected by auditory deprivation are critical to developing closer to typical speech outcomes. Moreover, the findings suggest that determination of the type of neural reorganization caused by auditory deprivation before implantation is valuable for predicting post-CI language outcomes for young children.

Outcomes and Time to Emergence of Auditory Skills After Cochlear Implantation of Children With Charge Syndrome.

OBJECTIVE: Review perioperative complications, benefits, and the timeframe over which auditory skills develop in children with CHARGE syndrome who receive a cochlear implant (CI). STUDY DESIGN: IRB-approved retrospective chart review of children with CHARGE syndrome who had at least 12 months of cochlear implant use. SETTING: Tertiary care children's hospital. PATIENTS: Twelve children, seven males and five females. Mean age implant = 3.5 years (1.7-8.2 yr); mean duration follow-up = 4.7 years (1.5-10.1 yr). INTERVENTION: Cochlear implantation. MAIN OUTCOME MEASURES: Auditory skills categorized into four levels, temporal bone imaging findings, perioperative complications, time to emergence of speech perception, expressive communication mode. RESULTS: All children imaged with magnetic resonance imaging had cochlear nerve deficiency in at least one ear. Speech awareness threshold improved with the CI compared with aided preoperative in 83% of children, with means of 51.7 dB SAT preoperative and 27.1 dB with the CI (p ≤ 0.002). Overall, four children improved to auditory Level 2 (improved detection), three obtained Level 3 (closed-set speech perception), and three had open-set speech perception with their CIs (Level 4) that was first evident at 3.5, 3.3, and 0.8 years postimplant testing. Two children had minimal or limited improvement. One child with hypoplasia of the cochlear nerve obtained open-set levels. CONCLUSION: Auditory skills may develop slowly in children with CHARGE syndrome who receive a CI but most can achieve at least improved detection. In our series, half acquired some speech perception ability. Cochlear nerve deficiency is frequent, but should not be a contraindication to implantation.

Management of acute complicated mastoiditis at an urban, tertiary care pediatric hospital.

OBJECTIVES/HYPOTHESIS: To describe the presentation and management of acute complicated mastoiditis in children. STUDY DESIGN: Retrospective case series. METHODS: An analysis of pediatric patients with acute complicated mastoiditis treated at an urban, tertiary care children's hospital from 2007 to 2014 was performed. RESULTS: Forty-eight patients presented with a total of 67 complications of acute mastoiditis. Mean age at presentation was 4.8 years (range = 0.1-15.3 years). The most common complications were subperiosteal abscess (n = 22, 45.8%), epidural abscess (n = 16, 33.3%), and sigmoid sinus thrombosis (n = 14, 29.2%). The most common pathogens isolated included Streptococcus pneumoniae (n = 14, 29.2%) and group A streptococcus (n = 10, 20.8%). Multidrug resistance was not associated with complication type. Surgical management included myringotomy ± tympanostomy tube placement in 46 (95.8%) patients (the only surgery in 10), drainage of subperiosteal abscess without mastoidectomy in 18 (37.5%) patients, and mastoidectomy in 21 (43.8%) total patients. Patients presenting with intracranial complications were the most likely to undergo a mastoidectomy. Anticoagulation was used in the management of nine of 14 (64.3%) patients presenting with sigmoid sinus thrombosis. Neurosurgical interventions (n = 7, 14.6% patients) were primarily performed to manage increased intracranial pressure. CONCLUSIONS: Subperiosteal abscess was the most common complication of acute mastoiditis, and when occurring as the sole complication was successfully managed with antibiotics and surgical intervention that did not include mastoidectomy. Epidural abscess and sigmoid sinus thrombosis were more prevalent than reported in prior series and were managed more aggressively. These patients were more likely to need neurosurgical interventions. LEVEL OF EVIDENCE: 4. Laryngoscope, 127:2321-2327, 2017.

Cochlear implantation of solid organ transplant patients receiving immunosuppressive therapy.

OBJECTIVE: To evaluate the incidence of infectious complications and healing problems in cochlear implant (CI) patients receiving immunosuppressive therapy following solid organ transplant. STUDY DESIGN: IRB-approved retrospective chart review of implanted patients. SETTING: Tertiary care children's hospital. METHODS: Seven patients of the more than 1000 implanted during the time period between 1991 and 2014 underwent cochlear implantation while on immunosuppressive therapy after having received a solid organ transplant. Their charts were reviewed for demographic and medical information pre- and post-implantation. The 4 males and 3 females ranged in age at CI from 2.4 to 18.8 years, with a mean of 9.0 years. Postimplant follow-up averaged 3.9 years (range: 0.1-13.1). Main outcome measures were occurrence of wound healing, infectious complications, whether open-set word recognition was achieved, and communication mode(s). RESULTS: No wound infections or delayed healing, mastoiditis, or bacterial meningitis occurred after cochlear implantation. All seven patients had received at least one pneumococcal vaccination prior to implantation. Five of 6 (83%) developed open-set speech perception, of whom 4 (67%) use only oral communication. CONCLUSION: In our series of patients receiving immunosuppressive therapy following solid organ transplantation, none developed wound healing or infectious complications after cochlear implantation. History of solid organ transplantation alone should not be a contraindication to cochlear implant candidacy which would deprive the child of the potential benefits of hearing, including language development.

Magnetic Resonance Imaging of Cochlear Implant Recipients.

OBJECTIVE: Determine the diagnostic usefulness of postimplantation 1.5 T magnetic resonance imaging (MRI) and review magnet-related MRI complications. STUDY DESIGN: Retrospective chart review with additional review of MRIs. SETTING: Tertiary care children's hospital. PATIENTS: Twelve patients who underwent MRI after receiving a cochlear implant (CI). INTERVENTION: One or more episodes of 1.5 T MRI with CI in place. MAIN OUTCOME MEASURES: Occurrence of magnet-related complications; whether imaging was clinically useful. RESULTS: The 12 patients underwent 23 episodes of MRI, including 13 episodes in 11 patients (18 ears) during which a magnet was present and 17 studies were obtained. Complications related to the magnet occurred during 4 of the 13 imaging episodes (30.8%), all during body or spine studies. Magnet torsion with reversal of polarity occurred in three devices; reduced magnet strength in one; and displacement of the magnet from its housing in one. One patient required surgical magnet replacement, whereas other headpiece retention problems were resolved without surgery. All studies but one brain with bilateral magnets were clinically useful. CONCLUSIONS: CI patients who undergo MRI with a magnet in situ may experience complications, especially when imaged below the head. Most complications may be resolved without surgery. Diagnostic usefulness of non-cranial MRI is not likely to be limited by presence of the magnet, while a magnet may prevent clinically useful brain imaging. Obtaining MRI with the magnet in situ avoids the cost and risks associated with multiple surgeries to remove and replace the magnet or the entire implant.

Preoperative imaging of sensorineural hearing loss in pediatric candidates for cochlear implantation.

Cochlear implantation is the only U.S. Food and Drug Administration-approved treatment for children with marked bilateral sensorineural hearing loss. It provides auditory benefits that range from simple sound detection to substantial word understanding. Improved hearing through cochlear implantation has been demonstrated to enhance the rate of language acquisition, enable development of spoken language, and advance literacy in deaf children. Magnetic resonance imaging and computed tomography both have roles in the preoperative assessment of inner-ear abnormalities, cochlear nerve deficiency, and variant anatomy that may affect the decision to implant and the prognosis for auditory improvement and increase the risk for complications. Most cochlear abnormalities may be successfully treated with cochlear implantation, but the presence of a cochlear malformation may increase the risk for intraoperative cerebrospinal fluid leakage and postoperative bacterial meningitis. Eighth-nerve deficiency correlates with poor auditory outcomes and may affect eligibility for cochlear implantation. Another important consideration for implantation is the presence of labyrinthitis ossificans in some children with deafness resulting from bacterial meningitis, which may cause obstruction that limits electrode insertion. Anatomic variations of the facial nerve or middle-ear cavity, which are more common in syndromic patients, may also affect the surgical approach and make implantation difficult.

Auditory-cognitive training improves language performance in prelingually deafened cochlear implant recipients.

OBJECTIVES: Phonological and working memory skills have been shown to be important for the development of spoken language. Children who use a cochlear implant (CI) show performance deficits relative to normal hearing (NH) children on all constructs: phonological skills, working memory, and spoken language. Given that phonological skills and working memory have been shown to be important for spoken language development in NH children, we hypothesized that training these foundational skills would result in improved spoken language performance in CI-using children. DESIGN: Nineteen prelingually deafened CI-using children aged 4- to 7-years-old participated. All children had been using their implants for at least one year and were matched on pre-implant hearing thresholds, hearing thresholds at study enrollment, and non-verbal IQ. Children were assessed on expressive vocabulary, listening language, spoken language, and composite language. Ten children received four weeks of training on phonological skills including rhyme, sound blending, and sound discrimination and auditory working memory. The remaining nine children continued with their normal classroom activities for four weeks. Language assessments were repeated following the training/control period. RESULTS: Children who received combined phonological-working memory training showed significant gains on expressive and composite language scores. Children who did not receive training showed no significant improvements at post-test. On average, trained children had gain scores of 6.35 points on expressive language and gain scores of 6.15 points whereas the untrained children had test-retest gain scores of 2.89 points for expressive language and 2.56 for composite language. CONCLUSION: Our results suggest that training to improve the phonological and working memory skills in CI-using children may lead to improved language performance.

Usher syndrome: characteristics and outcomes of pediatric cochlear implant recipients.

OBJECTIVE: To evaluate the characteristics and outcomes of pediatric cochlear implant recipients diagnosed with Usher syndrome (US). STUDY DESIGN: Retrospective study of consecutive pediatric cochlear implant recipients (1991-2010). SETTING: Tertiary care children's hospital. PATIENTS: Children who received a cochlear implant who were diagnosed with US either before or after implantation. MAIN OUTCOME MEASURES: Electroretinography and ophthalmologic findings, cochlear anatomy based on preoperative imaging, age of independent ambulation, age at implantation, speech perception level, and communication method. RESULTS: Approximately 26 (3.7%) of 712 cochlear implant recipients were diagnosed with US based on the results of electroretinography and/or genetic testing. Preoperative imaging revealed no evidence of cochlear malformations. Average age of independent ambulation was 21.9 months (range, 12-30). Average age at implantation was 3.3 years (range, 6 mo to 11.6 yr). Mean follow-up after implantation was 7.8 years (range, 10 mo to 15.6 yr). Open-set speech perception was present in 92% of children, with use of a primarily oral communication mode in 69.2%. CONCLUSION: In this large series of patients with the diagnosis of US who have undergone cochlear implantation, CT and MRI imaging were normal. Significant delay in independent ambulation was present in this population secondary to abnormal vestibular function associated with US Type I. A majority of children developed significant open-set speech perception and oral communication skills. Implantation of US children provides them with the opportunity to develop useful hearing and oral communication.

Pediatric cochlear implantation of children with eighth nerve deficiency.

OBJECTIVE: To evaluate the auditory outcomes of children implanted in an ear with eighth nerve hypoplasia or aplasia and to determine whether preoperative characteristics are predictive of auditory performance achieved. STUDY DESIGN: retrospective case review. SETTING: tertiary care medical center. PATIENTS: ten children implanted in an ear with eighth nerve hypoplasia or aplasia, as determined by high resolution magnetic resonance imaging. MAIN OUTCOME MEASURES: Neural response test measurements, detection and speech awareness thresholds, Meaningful Auditory Integration Scale scores, as well as speech perception level achieved. RESULTS: Post-implantation, three children demonstrated little to no detection of sound, three had improved detection and awareness of environmental sounds, one developed closed-set speech perception and spoken language, and three developed open-set speech perception and spoken language. No imaging findings appeared related to outcomes. Significantly better implant detection thresholds and Meaningful Auditory Integration Scale scores were found in children who had preoperative aided auditory detection (p's ≤ 0.02-0.05). CONCLUSION: Some children with eighth nerve hypoplasia or aplasia may derive significant benefit from a cochlear implant. In our study high resolution magnetic resonance imaging was more sensitive than high resolution computer tomography in detecting neural deficiency. However, no imaging findings were predictive of auditory performance level achieved post-implantation.