The Role of Motion Sensitivity and Headaches on Vestibular Rehabilitation Outcomes in Pediatric Vestibular Migraines.

OBJECTIVE: To determine the utility of the motion sensitivity quotient (MSQ) in diagnosing pediatric vestibular migraine (VM) and to characterize the role of motion sensitivity and headache control on vestibular rehabilitation (VR) outcomes in pediatric VM. STUDY DESIGN: Retrospective cohort analysis. SETTING: Pediatric tertiary referral center. PATIENTS: Children (≤18 years old) with dizziness who completed vestibular testing from January 2016 to August 2022, diagnosed with either VM or another vestibular disorder. INTERVENTIONS: VR, which included MSQ testing. MAIN OUTCOME MEASURES: Initial MSQ, number and duration of vestibular physical therapy (PT) sessions, PT goals met, and posttreatment MSQ. RESULTS: Two hundred fifty-seven patients met study criteria. MSQ was not a reliable diagnostic marker in pediatric VM as there was no difference in initial MSQ between VM and non-VM patients (9.4 vs. 7.8 in non-VM, p = 0.014). Both VM (n = 116) and non-VM (n = 141) patients demonstrated significant improvement in MSQ after VR (p = 0.004). However, VM patients tended to be less likely to meet at least one PT goal (60 vs. 77% in non-VM, p = 0.016, d = 0.37), although not significant. VM patients with more frequent headaches had significantly higher initial MSQ (p = 0.008). VM patients with more frequent headaches or higher initial MSQ tended to require increased number and longer duration of VR (small/medium effect size although not statistically significant after Bonferroni correction). CONCLUSION: VR is an effective treatment for both VM and non-VM pediatric patients. VM patients, especially those with severe motion sensitivity or poorly controlled headaches, may be less responsive to VR and may require increased frequency and duration of VR. Our findings propose the importance of counseling pediatric patients with severe motion sensitivity or uncontrolled migraines regarding realistic expectations of their VR course.

The Girl With the Bleeding Earlobe Mass.

ABSTRACT: Pilomatrixomas, also known as epithelioma calcificans, are benign tumors of hair follicle matrix cells that are often mistaken for other lesions, especially cutaneous abscesses. We report an illustrative case in which a teenage girl developed a red, swollen earlobe that required multiple care visits and interventions until definitive diagnosis and treatment were provided. Although the lesion was initially treated as an abscess, it continued to progress in size and discomfort. The correct diagnosis was established after imaging and complete excision with pathologic examination. Ultimately, our patient was subjected to avoidable procedures that carried the risk of potentially negative cosmetic sequelae before the proper intervention. Although abscesses are common, it is important for clinicians to avoid incision and drainage of lesions, unless the diagnosis is certain.

Socioeconomic Disparities in Pediatric Single-Sided Deafness.

OBJECTIVE: To explore socioeconomic disparities in pediatric single-sided deafness (SSD) treatment. STUDY DESIGN: Retrospective chart review. SETTING: Tertiary referral academic center. METHODS: The charts of 190 pediatric patients with SSD were reviewed for demographic and clinical characteristics. Socioeconomic variables included race and insurance status. ZIP codes were used to obtain additional socioeconomic data from the American Community Survey, including mean and median income, percentage of families below the poverty level, and employment status. Socioeconomic status (SES) was classified by insurance status and income. Treatment outcomes were analyzed by socioeconomic variables. RESULTS: There were 105 males and 85 females with a mean follow-up of 55.2 months and a mean age at diagnosis of 4.4 years. Sixty-three percent of children received treatment at last follow-up. Thirty-five percent of children had public insurance and 65% had private insurance. Treatment rates were similar in the private and public insurance groups (60.6% vs 66.7%, P = .42), but device type was different between groups (P = .02). Consistent device use was associated with private insurance (47.5% vs 38.9%, P = .003) and high SES (94.4% vs 80%, P = .04) on univariate but not on multivariate analysis. Aided audiometry results were similar between SES groups. No association was found between sex, race, income level, poverty level, or employment status and treatment outcomes. CONCLUSION: Insurance type and SES were not associated with SSD treatment outcomes in children, although device use may be higher in children with private insurance and higher SES. Further research should focus on strategies to reduce barriers to treatment and improve adherence.

Imaging findings in pediatric single-sided deafness and asymmetric hearing loss.

OBJECTIVE: To examine the imaging findings on computer tomography (CT) and magnetic resonance imaging (MRI) in pediatric single-sided deafness (SSD) and asymmetric hearing loss (ASH). METHODS: The medical records of 189 pediatric patients with SSD and ASH were retrospectively reviewed, and imaging findings were compared. SSD was defined as unilateral profound hearing loss and contralateral normal hearing ear. In the ASH group, ASHw was defined as the worse hearing ear with profound hearing loss, while ASHb was defined as the better hearing ear with mild-moderate hearing loss. RESULTS: There were 170 patients with SSD and 19 patients with ASH. In the SSD group, 83 patients (48.8%) had imaging findings associated with hearing loss. In the ASH group, such imaging findings were found in six (31.6%) of the ASHw and in five (26.3%) of the ASHb ears. The most common finding in the SSD group was cochlear nerve deficiency (50.6%), followed by cochlear dysplasia (39.8%) and enlarged vestibular aqueduct (26.5%). In the ASH groups, cochlear dysplasia was seen in three (50%) of ASHw ears and in two (40%) of the ASHb ears, and enlarged vestibular aqueduct was seen in three (50%) of ASHw ears and in two (40%) of the ASHb ears. CONCLUSION: Imaging studies identified the etiology in half of the cases of SSD and in one-third of ASH patients. Our findings strongly support the use of imaging studies in the evaluation of pediatric SSD and ASH. LEVEL OF EVIDENCE: 4 Laryngoscope, 130:1007-1010, 2020.

International Pediatric Otolaryngology Group (IPOG) Consensus Recommendations: Congenital Cholesteatoma.

OBJECTIVE: To provide recommendations to otolaryngologists and allied physicians for the comprehensive management of children who present with signs and symptoms of congenital cholesteatoma. METHODS: A two-iterative Delphi method questionnaire was used to establish expert recommendations by the members of the International Pediatric Otolaryngology Group, on the preoperative work-up, the perioperative considerations, and follow-up. RESULTS: Twenty-two members completed the survey, in 14 tertiary-care center departments representing 5 countries. The main consensual recommendations were: a precise otoscopic description of the quadrants involved, extensive audiological workup (bilateral tonal, vocal audiometry, and BERA), and a CT scan are required. Facial nerve monitoring and a combination of microscope and telescope are recommended for surgical removal. Clinical and audiological follow-up should be pursued yearly for at least 5 years. First MRI follow-up should be done at 18 months postoperatively if the removal violated the matrix. MRI follow-up duration depends on the initial extent of the cholesteatoma. CONCLUSION: The goal of preoperative and follow-up consensus from International Pediatric Otolaryngology Group participants is to help manage infants and children with congenital cholesteatoma. The operative techniques may vary, and experienced surgeons must perform these procedures.

Ocular Vestibular Evoked Myogenic Potentials: Normative Findings in Children.

BACKGROUND: To add to the limited body of literature on ocular vestibular evoked myogenic potential (oVEMP) responses in children and to assess a different montage for oVEMP recording. PURPOSE: To evaluate the characteristics of the oVEMP response in children and compare the results with that of a group of healthy adults. RESEARCH DESIGN: Prospective descriptive study from a tertiary referral center. STUDY SAMPLE: Twenty-two children (mean age = 6.3 yr, standard deviation = ±1.5, range = 3.5-8.9 yr) were recruited from families whose parent(s) were employed by the Cincinnati Children's Hospital Medical Center (CCHMC). Pediatric participants were categorized by age into three groups for data analysis. The comparison adult group of ten participants were members of the employee staff at CCHMC. DATA COLLECTION AND ANALYSIS: Audiometric assessment was completed in all participants. The latency, amplitude, and threshold of the oVEMP responses were recorded using a modified electrode montage with reference at the chin and compared between the pediatric and adult participants. RESULTS: All participants completed testing and had bilateral measurable oVEMP responses using a 105-dB nHL, 500-Hz tone burst stimulus. Comparison between right and left ears across all participants for each oVEMP characteristic found no statistically significant difference. oVEMP testing showed no significant differences with respect to latency, amplitude, interaural amplitude asymmetry, and threshold of response as a function of age. CONCLUSIONS: oVEMP responses for ages ≥3 did not differ from responses in adults.

Vestibular pathology in children with enlarged vestibular aqueduct.

OBJECTIVES/HYPOTHESIS: To establish the prevalence of abnormal vestibular test findings in children with enlarged vestibular aqueduct (EVA) and determine if these findings correlate with clinical symptoms, radiographic findings (EVA size and laterality), audiometric findings, and genetic testing in these patients. STUDY DESIGN: Prospective cohort. METHODS: Patients 3 to 12 years of age with hearing loss and imaging findings consistent with EVA treated at our tertiary care institution were sequentially enrolled from 2009 to 2011. The following six outcome measurements were analyzed: audiometric findings, EVA laterality, temporal bone measurements, genetic testing, vestibular testing (cervical-evoked myogenic potentials, posturography, rotational chair, and calorics), and vestibular symptoms. RESULTS: Twenty-seven patients with EVA (mean age 9.2 years, 48% female) were enrolled in and completed the study. Vertigo was reported in six patients. Twenty-four of 27 (89%) had at least one abnormal vestibular test result. Midpoint and operculum size correlated with directional preponderance (P = .042 and P = .032, respectively). Also, high-frequency pure tone average (HFPTA) correlated with unilateral weakness (P = .002). Walking at a later age correlated with abnormal posturography results. There was no correlation between EVA laterality and vestibular test findings. CONCLUSION: We found a high rate of vestibular pathology in children with EVA; however, the prevalence of abnormal vestibular test findings in this patient population was not correlated with vestibular symptoms. Enlarged vestibular aqueduct size, HFPTA, and walking at a later age were correlated with abnormal vestibular test findings. In view of these results, it may be prudent to consider vestibular testing in children with these clinical characteristics. LEVEL OF EVIDENCE: 2b. Laryngoscope, 126:2344-2350, 2016.

High-frequency sensorineural hearing loss in children.

OBJECTIVES/HYPOTHESIS: Determine the prevalence of high-frequency sensorineural hearing loss (HFSNHL) in our hearing loss population and a diagnostic algorithm for these patients. STUDY DESIGN: Retrospective case series. METHODS: We identified patients diagnosed with sensorineural hearing loss (SNHL) at our pediatric tertiary care institution from 1981 to 2010. Based on audiometric profiles, these patients were subdivided into those with a flat SNHL configuration and those with HFSNHL. Imaging and genetic testing data and data regarding age at diagnosis, laterality, and risk factors were obtained for both groups. Comparisons were then made between the two groups. RESULTS: Of 2,867 patients included in the study, 7.6% had HFSNHL. Age at diagnosis was significantly higher in HFSNHL patients (8.3 years vs. 6.1 years; P < .0001). These patients also had a significantly higher proportion of unilateral versus bilateral loss (49.1% vs. 26.1%; P < .0001); unilateral losses were also less severe. Genetic testing showed no significant difference between groups in the proportion of patients tested or in those who tested positive. Similarly, imaging data revealed no difference in the proportion of patients tested in the two groups; however, overall diagnostic yield was significantly higher in flat SNHL patients (29.5% vs.17.3; P = .02). CONCLUSIONS: The positive predictive value of simple genetic testing is similar to that of imaging studies. However, given cost differences between genetic testing and imaging, it is prudent to perform genetic testing as the initial diagnostic test. Determination of whether high-throughput, multigene diagnostic platforms offer an added benefit in the evaluation of children requires further study. LEVEL OF EVIDENCE: 4. Laryngoscope, 126:1236-1240, 2016.

Diagnostic Evaluation of Children with Sensorineural Hearing Loss.

The cause of pediatric sensorineural hearing loss is diverse, comprising genetic, acquired, and idiopathic conditions. Identifying the specific cause requires that children undergo thorough otolaryngologic and audiometric evaluations, which generally include laboratory tests and temporal bone imaging studies. Clinical genetics and ophthalmologic consultations are also frequently warranted. A sequential diagnostic approach has been shown to be both prudent and cost-effective. Although a definitive cause is being established, amplification and a comprehensive treatment strategy should be initiated to ensure that developmental, auditory, and speech and language delays are minimized.

Molecular genetics of MARVELD2 and clinical phenotype in Pakistani and Slovak families segregating DFNB49 hearing loss.

Pathogenic mutations of MARVELD2, encoding tricellulin, a tricelluar tight junction protein, cause autosomal recessive non-syndromic hearing loss (DFNB49) in families of Pakistan and Czech Roma origin. In fact, they are a significant cause of prelingual hearing loss in the Czech Roma, second only to GJB2 variants. Previously, we reported that mice homozygous for p.Arg497* variant of Marveld2 had a broad phenotypic spectrum, where defects were observed in the inner ear, heart, mandibular salivary gland, thyroid gland and olfactory epithelium. The current study describes the types and frequencies of MARVELD2 alleles and clinically reexamines members of DFNB49 families. We found that MARVELD2 variants are responsible for about 1.5 % (95 % CI 0.8-2.6) of non-syndromic hearing loss in our cohort of 800 Pakistani families. The c.1331+2T>C allele is recurrent. In addition, we identified a novel large deletion in a single family, which appears to have resulted from non-allelic homologous recombination between two similar Alu short interspersed elements. Finally, we observed no other clinical manifestations co-segregating with hearing loss in DFNB49 human families, and hypothesize that the additional abnormalities in the Marveld2 mutant mouse indicates a critical non-redundant function for tricellulin in other organ systems.

Codeine-related adverse drug reactions in children following tonsillectomy: a prospective study.

OBJECTIVES/HYPOTHESIS: To prospectively determine factors associated with codeine's adverse drug reactions (ADRs) at home in a large homogenous population of children undergoing outpatient tonsillectomy. STUDY DESIGN: Prospective, genotype blinded, observational study with a single group and repeated ADR measures documented by parents at home. METHODS: A total of 249 children 6 to 15 years of age scheduled for tonsillectomy were enrolled. The primary outcome was number of daily codeine-related ADRs. We examined the number and type of ADR by race and by days and further modeled factors potentially associated with ADR risk in a subcohort of white children. Sedation following a dose of codeine was a secondary outcome measure. Parents recorded their children's daily ADRs and sedation scores during postoperative days (POD) 0 to 3 at home. RESULTS: Diaries were returned for 134 children, who were given codeine. A total of 106 (79%) reported at least one ADR. The most common ADRs were nausea, lightheadedness/dizziness for white children and nausea, and vomiting for African American children. In a subcohort of white children ≤ 45 kg, increased ADR risk was associated with the presence of one or more full function CYP2D6 alleles (P < 0.001), POD (P < 0.001), and sex (P = 0.027). Increased pain intensity (P = 0.009) and PODs 0 and 1 (P = 0.001) contributed to a higher sedation risk. Neither obstructive apnea nor predicted CYP2D6 phenotype were associated with sedation risk. CONCLUSIONS: Our results provide evidence that multiple factors are associated with codeine-related ADRs and support the FDA recommendation to avoid codeine's routine use following tonsillectomy in children.

Genetic analysis through OtoSeq of Pakistani families segregating prelingual hearing loss.

OBJECTIVE: To identify the genetic cause of prelingual sensorineural hearing loss in Pakistani families using a next-generation sequencing (NGS)-based mutation screening test named OtoSeq. STUDY DESIGN: Prospective study. SETTING: Research laboratory. SUBJECTS AND METHODS: We used 3 fluorescently labeled short tandem repeat (STR) markers for each of the known autosomal recessive nonsyndromic (DFNB) and Usher syndrome (USH) locus to perform a linkage analysis of 243 multigenerational Pakistani families segregating prelingual hearing loss. After genotyping, we focused on 34 families with potential linkage to MYO7A, CDH23, and SLC26A4. We screened affected individuals from a subset of these families using the OtoSeq platform to identify underlying genetic variants. Sanger sequencing was performed to confirm and study the segregation of mutations in other family members. For novel mutations, normal hearing individuals from ethnically matched backgrounds were also tested. RESULTS: Hearing loss was found to co-segregate with locus-specific STR markers for MYO7A in 32 families, CDH23 in 1 family, and SLC26A4 in 1 family. Using the OtoSeq platform, a microdroplet PCR-based enrichment followed by NGS, we identified mutations in 28 of the 34 families including 11 novel mutations. Sanger sequencing of these mutations showed 100% concordance with NGS data and co-segregation of the mutant alleles with the hearing loss phenotype in the respective families. CONCLUSION: Using NGS-based platforms like OtoSeq in families segregating hearing loss will contribute to the identification of common and population-specific mutations, early diagnosis, genetic counseling, and molecular epidemiology.

Performance evaluation of the next-generation sequencing approach for molecular diagnosis of hereditary hearing loss.

OBJECTIVE: To evaluate the performance of a next-generation sequencing (NGS)-based targeted resequencing genetic test, OtoSeq, to identify the sequence variants in the genes causing sensorineural hearing loss (SNHL). STUDY DESIGN: Retrospective study. SETTING: Tertiary children's hospital. SUBJECTS AND METHODS: A total of 8 individuals presenting with prelingual hearing loss were used in this study. The coding and flanking intronic regions of 24 well-studied SNHL genes were enriched using microdroplet polymerase chain reaction and sequenced on an Illumina HiSeq 2000 sequencer. The filtered high-quality sequence reads were mapped to reference sequence, and variants were detected using NextGENe software. RESULTS: A total of 1148 sequence variants were detected in 8 samples in 24 genes. Using in-house developed NGS data analysis criteria, we classified 810 (~71%) of these variants as potential true variants that include previously detected pathogenic mutations in 5 patients. To validate our strategy, we Sanger sequenced the target regions of 5 of the 24 genes, accounting for about 29.2% of all target sequence. Our results showed >99.99% concordance between NGS and Sanger sequencing in these 5 genes, resulting in an analytical sensitivity and specificity of 100% and 99.997%, respectively. We were able to successfully detect single base substitutions, small deletions, and insertions of up to 22 nucleotides. CONCLUSION: This study demonstrated that our NGS-based mutation screening strategy is highly sensitive and specific in detecting sequence variants in the SNHL genes. Therefore, we propose that this NGS-based targeted sequencing method would be an alternative to current technologies for identifying the multiple genetic causes of SNHL.

Minimal access and standard cochlear implantation: a comparative study.

OBJECTIVE: To compare the operative times and complications between patients who underwent minimal access cochlear implantation and standard technique cochlear implantation. METHODS: Patients who underwent unilateral cochlear implantation by a single surgeon from 2001 to 2010. The minimal access technique of an approximately 2.5-3 cm post-auricular incision with creation of subperiosteal pocket for the device was compared to the longer standard "S" incision into the scalp (~8-10 cm) with bone well creation and suture fixation. Outcomes include operative times and complications. RESULTS: There were 122 unilateral implants, 73 (59.8%) in the minimal access group and 49 (40.2%) in the standard group. Mean total time in the operating room was lower in the minimal access group compared to the standard group (200±31 vs. 255±49 min, p<.0001) as well as mean operative time (149.5±28 vs. 200±45 min, p<.0001 respectively). There were 17 complications in the entire cohort with 8 and 9 complications in the minimal and standard groups respectively. Of the 17 complications, 12 were surgical technique-specific. Although it appeared that there were higher rates of major, technique-specific, and overall complications in the standard access group, these differences did not reach statistical significance. CONCLUSIONS: Patients undergoing minimal access cochlear implantation require shorter operative times when compared to the standard access cochlear implantation. In addition, low complication rates are observed for major, technique-specific, and overall complications. Minimal access cochlear implantation may be considered an equivalent and potentially superior technique.

Characteristics of children diagnosed as having coagulopathies following posttonsillectomy bleeding.

OBJECTIVE: To determine the prevalence of coagulopathy among children presenting with posttonsillectomy bleeding (PTB) and describe risk factors that could indicate the presence of occult coagulopathy. DESIGN: Retrospective medical chart review. SETTING: Tertiary-care pediatric hospital. PATIENTS: The study population comprised 182 patients presenting with PTB from January to December 2007. MAIN OUTCOME MEASURES: Demographics, laboratory studies, type of intervention, transfusion status, need for hematology consultation, type of coagulopathy, and disposition were recorded. RESULTS: There were 216 emergency department (ED) encounters for PTB. The mean age of the patients was 8.4 years, and 56% were male and 79% were white. Patients presented on mean postoperative day 5.9. Of the 182 children, 34 (19%) presented with abnormally elevated prothrombin time, activated partial thromboplastin time, or platelet function assays (PFAs) for both adenosine diphosphate and epinephrine. Eight patients (4%) ultimately were diagnosed as having a coagulopathy. Differences in mean age (P = .23), sex (P = .47), race (P = .76), number of days posttonsillectomy (P = .34), and higher ED visit frequency (P = .06) between the coagulopathic and noncoagulopathic children were not statistically significant. Coagulopathic children had significantly higher mean activated partial thromboplastin time (P < .001), PFA for adenosine diphosphate (P < .001), and PFA for epinephrine (P = .001). Of the 8 coagulopathic children, 3 (38%) presented with a history of oral bleeding and a normal physical examination. CONCLUSIONS: In children presenting with PTB, activated partial thromboplastin time and PFA studies and hematology consultations are helpful in identifying occult coagulopathies. The definition of PTB should be broadened to include children with any history of oral bleeding, regardless of examination findings.

Genetic mutations and aminoglycoside-induced ototoxicity in neonates.

OBJECTIVE: Mutations in the 12S rRNA gene have been associated with aminoglycoside-induced ototoxicity. Our objective was to study the relationship of these mutations in neonates, duration of aminoglycoside exposure, and other known risk factors to the presence of hearing loss. STUDY DESIGN: Prospective case-cohort study. SETTING: Three neonatal intensive care units (NICUs) in Cincinnati, OH. SUBJECTS AND METHODS: We studied a population of premature, low-birth-weight (< 2500 g) infants admitted to one of three ICUs. Demographic, genetic, clinical, and audiometric data were collected, and the prevalence of 12S rRNA mutations was calculated. RESULTS: Of the 436 patients enrolled in the study, 378 were exposed to gentamicin during their ICU stay. Mutations in the 12S rRNA gene were identified in four patients (0.9%), all of whom received gentamicin. Of the cohort, 256 patients (60%) received a complete audiometric assessment; 39 failed their initial hearing assessment. Only one of these patients had a 12S rRNA mutation. Of these 39 patients, the mean birth weight (1645 g vs 1306 g) was significantly less than the birth weight of those infants who passed their initial hearing screening. Definitive hearing assessment for those who failed showed no significant differences, however. CONCLUSION: The prevalence of 12S rRNA mutations related to aminoglycoside ototoxicity in our study population was approximately one percent. Most patients with this mutation and aminoglycoside exposure showed no evidence of hearing loss. Low birth weight was one risk factor related to the presence of failing a hearing assessment.

H1N1 influenza A presenting as bacterial tracheitis.

Six cases of bacterial tracheitis (BT) occurring early in the 2009 flu season have been isolated in conjunction with the H1N1 strain of influenza A (H1N1). No previous H1N1 cases have presented as BT in the literature to date. We would like to discuss viral coinfection in BT patients and how this new strain may affect the rate and type of presentation encountered. The life-threatening potential of BT and the pandemic proportion of H1N1 highlight a possibly dangerous combination that should be recognized by the otolaryngology community. In hospitalized patients with presumed BT, consideration should be given to routine H1N1 testing and the addition of antiviral medication when indicated as this entity is further investigated.

High-throughput detection of mutations responsible for childhood hearing loss using resequencing microarrays.

BACKGROUND: Despite current knowledge of mutations in 45 genes that can cause nonsyndromic sensorineural hearing loss (SNHL), no unified clinical test has been developed that can comprehensively detect mutations in multiple genes. We therefore designed Affymetrix resequencing microarrays capable of resequencing 13 genes mutated in SNHL (GJB2, GJB6, CDH23, KCNE1, KCNQ1, MYO7A, OTOF, PDS, MYO6, SLC26A5, TMIE, TMPRSS3, USH1C). We present results from hearing loss arrays developed in two different research facilities and highlight some of the approaches we adopted to enhance the applicability of resequencing arrays in a clinical setting. RESULTS: We leveraged sequence and intensity pattern features responsible for diminished coverage and accuracy and developed a novel algorithm, sPROFILER, which resolved >80% of no-calls from GSEQ and allowed 99.6% (range: 99.2-99.8%) of sequence to be called, while maintaining overall accuracy at >99.8% based upon dideoxy sequencing comparison. CONCLUSIONS: Together, these findings provide insight into critical issues for disease-centered resequencing protocols suitable for clinical application and support the use of array-based resequencing technology as a valuable molecular diagnostic tool for pediatric SNHL and other genetic diseases with substantial genetic heterogeneity.

Neuroanatomic differences in children with unilateral sensorineural hearing loss detected using functional magnetic resonance imaging.

OBJECTIVE: Functional magnetic resonance imaging (fMRI) provides information about neuronal excitation by measuring changes in cerebral hemodynamics. This study used fMRI to compare neuroanatomic activation patterns in children with unilateral sensorineural hearing loss (USNHL) with the neuroanatomic activation patterns in normally hearing individuals. DESIGN: Patients were presented with narrowband noise and speech-in-noise tasks while undergoing fMRI of the brain. In the narrowband noise task, 5 chirps at center frequencies of 250 Hz, 500 Hz, 1 kHz, 2 kHz, and 4 kHz were presented monaurally for 1 second in a randomized order to children in both groups. In the speech-in-noise task, Bamford-Kowal-Bench (BKB) sentences were presented over 4-talker babble to both ears, and scans were acquired after each stimulus. We compared fMRI data across groups using independent component analysis and Bayesian (hierarchical) linear models. SETTING: Tertiary referral center. PATIENTS: Twelve children with USNHL and 23 normally hearing controls. INTERVENTIONS: Perform fMRI while subject listens to narrowband and speech-in-noise tasks. MAIN OUTCOME MEASURES: Neuroanatomic differences in fMRI. RESULTS: In the narrowband noise task, children with USNHL had less activation of auditory areas and failed to activate auditory association areas and attention networks compared with normally hearing controls. In the speech-in-noise task, children with USNHL activated only secondary auditory processing areas in the left hemisphere, while controls activated these areas bilaterally. Children with right-sided USNHL failed to activate attention areas that were activated in controls and in children with left-sided USNHL. Only children with left-sided USNHL activated bilateral visual association areas. CONCLUSIONS: Results show significant differences in the cortical processing of sound between children with severe to profound USNHL and normally hearing children. These differences may account for the functional auditory problems that children with USNHL experience.