Differences in Hearing Devices and Speech Therapy Utilization Between Children With Permanent Unilateral Versus Bilateral Hearing Loss.

OBJECTIVES: In this study, we aimed to describe differences in diagnosis and both auditory and speech/language intervention utilization between children with permanent unilateral hearing loss as compared with bilateral hearing loss. DESIGN: A retrospective cohort study was performed of children evaluated in a multidisciplinary hearing loss clinic at a tertiary care pediatric hospital. Children aged 0 to 18 years with either permanent unilateral or bilateral hearing loss were included. RESULTS: One hundred fourteen children with unilateral hearing loss and 268 children with bilateral hearing loss were studied for a total of 382 children. There were no demographic differences between children with permanent unilateral versus bilateral hearing loss. Rates of newborn hearing screening and referred screening results were similar between those with unilateral and bilateral hearing loss. Despite similar rates of referred newborn hearing screening, those with bilateral hearing loss were diagnosed at a younger age (mean 3.6 years, SD 3.8 years) as compared with those with unilateral hearing loss (mean 5.0 years, SD 4.2 years). Children with unilateral hearing loss had similar severity of hearing loss in their poorer hearing ear as compared with children with bilateral hearing loss, yet they were significantly less likely to be fitted with hearing devices (53% versus 78%) or receive speech/language therapy (36% versus 54%) as compared with children with bilateral hearing loss. Multivariate analysis found that bilateral hearing loss and earlier age of hearing loss diagnosis were associated with hearing device use. CONCLUSIONS: Early diagnosis and intervention for childhood hearing loss have a significant impact on a child's educational success and social relationships. However, little is known about differences in diagnosis and resource utilization between children with permanent unilateral hearing loss versus bilateral hearing loss. Children with unilateral hearing loss were diagnosed at a later age and were less likely to utilize hearing devices or speech/language therapy compared with those with bilateral hearing loss, despite having similar severity of hearing loss in the poorer hearing ear. There is a strong body of evidence that children with unilateral hearing loss have improved hearing outcomes with hearing devices, which suggests there is room for improvement in identifying unilateral hearing loss and providing adequate services to optimize educational success. However, speech therapy is generally implemented in response to language delays. Therefore, children with unilateral loss may have lower rates of language delays as compared with those with bilateral hearing loss, thereby explaining differences in speech therapy utilization.

Impact of Genetic Testing on Hearing Interventions.

OBJECTIVE: Clinical guidelines recommend genetic testing when evaluating congenital and late-onset sensorineural hearing loss (SNHL). Genetic diagnoses can provide parents additional information regarding anticipated hearing loss progression, comorbid conditions, and family planning. Additionally, obtaining a genetic diagnosis may increase parental acceptance of hearing loss and subsequent pursuit of intervention. This study evaluates the association between genetic diagnoses and hearing loss intervention. METHODS: We included children ages 0-18 years with SNHL who were hearing aid or cochlear implant candidates but non-users and underwent hearing-loss gene panel testing prior to initiating intervention. Univariate analyses were performed to identify predictors of hearing aid fitting or cochlear implantation. Multivariate logistic regression evaluated the impact of demographic and clinical factors on subsequent intervention. RESULTS: Of the 385 children with SNHL who underwent hearing loss gene panel testing, 111 were included. Median age was 7.5 years. 56% were underrepresented minorities, 71% were non-White, and 71% were publicly insured. Those found to have a genetic diagnosis were 4.6 times as likely to subsequently undergo intervention (p = 0.035). Additionally, bilateral hearing loss and earlier age of genetic testing were associated with increased likelihood of intervention. CONCLUSION: Up to half of children with SNHL are suspected to have an underlying genetic etiology. Children diagnosed with a genetic diagnosis are significantly more likely to subsequently utilize hearing aids or cochlear implantation. This provides additional support for clinical guidelines recommending genetic testing not only due to the impact of prognostication but also on treatment decision-making. LEVEL OF EVIDENCE: 4 Laryngoscope, 133:1982-1986, 2023.

Pandemics and pediatric otolaryngology.

The objective is to describe how the COVID pandemic changed the epidemiology and management of pediatric otolaryngologic diseases, which may influence clinical decision-making in the future. Many changes were made to the structure of healthcare delivery to minimize transmission of coronavirus. As a result, there was a widespread adoption of telehealth. Additionally, guidelines were published with new protocols for evaluation and management of common pediatric otolaryngologic conditions, which in many circumstances, delayed or replaced surgical intervention. Now, as we evaluate the impact of these clinical changes, we have gained new understanding about the pathophysiology of certain pediatric conditions, namely otitis media, for which upper respiratory infection exposure may play a larger role than previously thought. As we have altered practice patterns for common pediatric otolaryngologic conditions, we recognize that ongoing research may help us determine if surgical interventions have been overutilized in the past and help guide clinical practice guidelines moving forward.

Outcomes of an Early Childhood Hearing Screening Program in a Low-Income Setting.

IMPORTANCE: Early identification of childhood hearing loss through newborn hearing screening mitigates permanent speech, language, and developmental delays, but many children are lost to follow-up or develop postnatal hearing loss. Early childhood hearing screening programs may help identify these children, but evidence on their outcomes is limited. OBJECTIVE: To assess outcomes from a low-income, preschool-based hearing screening program and risk factors for hearing loss in this population. DESIGN, SETTING, AND PARTICIPANTS: A retrospective cohort study of 6820 children aged 2 to 6 years from urban, low-income public preschools who received hearing screening from July 1, 2015, to June 30, 2019, was performed using San Francisco Department of Public Health records. A multivariate logistic regression analyzed risk factors for hearing loss. Data analysis was conducted from January 14, 2020, to April 20, 2021. EXPOSURES: Annual single-visit, 2-tiered screening was implemented with conditioned play pure-tone audiometry (CPA) and distortion product otoacoustic emissions (OAEs). MAIN OUTCOMES AND MEASURES: Rates of successful screening, referred screening, loss to follow-up, and hearing loss. RESULTS: Of 6820 children (age, 2-6 years) screened, 3425 (50.2%) were boys, 15% were White/non-Hispanic, and 48% had English as the primary home language. A total of 403 (5.9%) children were referred for full medical or audiologic evaluation after 2-tiered CPA/OAE screening. Only 24 children were unable to complete both CPA and OAE testing for a screening completion rate of 99.6%. After medical evaluation, 114 of 403 children (28.3%) passed hearing rescreening and 55 (13.6%) were lost to follow-up. The prevalence of conductive hearing loss was 2.9% (n = 195), and the prevalence of sensorineural hearing loss was 0.2% (n = 13). Primary language, race and ethnicity, and sex were not associated with rates of referral or hearing loss. CONCLUSIONS AND RELEVANCE: The findings of this cohort study suggest that preschool-based screening programs can be a useful method to identify early childhood hearing loss and that teacher concerns are associated with final diagnostic hearing status.

Tracking Home Language Production and Environment in Children Who Are Deaf or Hard of Hearing.

OBJECTIVE: To use an automated speech-processing technology to identify patterns in sound environments and language output for deaf or hard-of-hearing infants and toddlers. STUDY DESIGN: Observational study based on a convenience sample. SETTING: Home observation conducted by tertiary children's hospital. METHODS: The system analyzed 115 naturalistic recordings of 28 children <3.5 years old. Hearing ability was stratified into groups by access to sound. Outcomes were compared across hearing groups, and multivariable linear regression was used to test associations. RESULTS: There was a significant difference in age-adjusted child vocalizations (P = .042), conversational turns (P = .022), and language development scores (P = .05) between hearing groups but no significant difference in adult words (P = .11). Conversational turns were positively associated with each language development measure, while adult words were not. For each hour of electronic media, there were significant reductions in child vocalizations (ß = -0.47; 95% CI, -0.71 to -0.19), conversational turns (ß = -0.45; 95% CI, -0.65 to -0.22), and language development (ß = -0.37; 95% CI, -0.61 to -0.15). CONCLUSIONS: Conversational turn scores differ among hearing groups and are positively associated with language development outcomes. Electronic media is associated with reduced discernible adult speech, child vocalizations, conversational turns, and language development scores. This effect was larger in children who are deaf or hard of hearing as compared with other reports in typically hearing populations. These findings underscore the need to optimize early language environments and limit electronic noise exposure in children who are deaf or hard of hearing.

Associations between demographic factors and pediatric otolaryngology access disparities in the COVID-19 era.

OBJECTIVES: To assess the impact of demographic factors and telehealth on access to pediatric otolaryngology care during the COVID-19 pandemic, as measured by attendance. METHODS: Retrospective, observational study of all referrals to pediatric otolaryngology at a single, tertiary care pediatric hospital system in the US. All referrals placed to pediatric otolaryngology from March through December 2020 were compared with referrals between March and December 2019. Data on patient demographics, date of referral, duration between referral and appointment, appointment type, and diagnosis acuity were collected. A multivariate linear regression was used to evaluate the impact of the patient age, ethnicity, language, insurance, diagnosis acuity, time to appointment, and appointment type on attendance. RESULTS: This study included 1988 referrals placed between March 16th-December 31st, 2020 and 3704 referrals placed between March 16th-December 31st, 2019. In 2020, attendance proportions were significantly higher at 72% compared to 62% during 2019 (p < 0.001). In 2020, there was a significantly shorter duration between referral and appointment, averaging 10 days as compared to 26 days in 2019 (p < 0.001). Overall, Black and Hispanic patients, children over the age of one, publicly insured patients, and those with longer wait times were less likely to attend their appointments. Primary language and use of telehealth did not predict attendance. CONCLUSION: Early evidence has found significant healthcare access and outcome disparities across ethnicities during the COVID pandemic. However, there is limited data evaluating the effect of demographic factors or telehealth on access to pediatric otolaryngology care. This study identifies age, race and insurance type as predictors of access to pediatric otolaryngologic care, as measured by attendance.

Obstructive Sleep Apnea: A Surgeon's Perspective.

Obstructive sleep apnea (OSA) is a complex medical disorder with significant impact on mortality, quality of life, and long-term cardiovascular outcomes. The apnea-hypopnea index does not correlate well with either quality-of-life measures or health outcomes, so other outcome measures must be evaluated in treatment of OSA. OSA can be successfully treated through behavioral, nonsurgical, and surgical methods with improvements in quality of life, morbidity, and mortality. Surgical intervention should be considered in patients who are noncompliant with or fail positive airway pressure use. As is true with PAP therapy, surgery for OSA improves mortality and symptoms of OSA even when the polysomnogram does not fully normalize.

A prospective study of patient-reported xerostomia-related outcomes after parotidectomy.

OBJECTIVE: There is a paucity of data on patient-reported outcome measures regarding xerostomia after parotidectomy surgery. Although salivary flow rates after parotidectomy have been previously studied, they do not correlate with subjective xerostomia. This study was designed to evaluate if unilateral parotidectomy increases patient-reported xerostomia. METHODS: A prospective cohort of patients undergoing unilateral partial, superficial, or total parotidectomy for benign or low-grade malignant pathology without postoperative radiation at a tertiary care academic center was studied. We analyzed patient-reported outcome measures of xerostomia using the Xerostomia Questionnaire (XQ) preoperatively and postoperatively. We compared pre- and postoperative cumulative and individual XQ scores using Wilcoxon signed-rank tests. We stratified patients by the weight in grams (g) of the parotid tissue excised, pathology, smoking status, and xerostomia-related medication use. RESULTS: Twenty-two adults with benign or low grade malignant unilateral parotid tumors were included. Postoperative questionnaires were completed at a median of 10.2 months (interquartile range [IQR] 8.6-11.9) after unilateral parotidectomy. Mean preoperative and postoperative cumulative XQ scores, on a 100-point scale, with higher scores representing worse symptoms, were 10.33 (95% CI: 4.46-16.20) and 10.54 (95% CI: 5.10-15.98), respectively, with a mean change of +0.21 (p = 0.472). There were no statistically significant changes in individual XQ symptom scores. Neither type of parotidectomy, resection specimens weighing over 10 g, smoking habits, xerostomia-related medication use, nor malignant pathology were associated with worse symptom scores. CONCLUSION: Based on these data, unilateral parotidectomy does not appear to definitely, or at least consistently, increase xerostomia per patient reporting. More extensive parotid resections are not associated with worse symptom scores. These data can help guide preoperative counseling and postoperative expectations for parotidectomy. LEVEL OF EVIDENCE: 2b.

Genetic Testing Leading to Early Identification of Childhood Ocular Manifestations of Usher Syndrome.

OBJECTIVES: Hearing-loss gene panel testing (HLGPT) is increasingly accessible as a first-line test in determining the etiology of sensorineural hearing loss (SNHL) in children. A major advantage of HLGPT is early identification of syndromic forms of SNHL, especially Usher syndrome, prior to the development of overt syndromic phenotype, which may impact management and counseling. Here, we describe early ocular findings in children with clinically non-syndromic SNHL identified by HLGPT as having two variants associated with Usher Syndrome. METHODS: A total of 184 children, ages 1 month - 15 years of age, evaluated at one tertiary pediatric children's hospital for clinically non-syndromic SNHL, underwent next-generation sequencing of 150 genes involved in hearing loss. Children with two variants in genes associated with Usher syndrome were referred for evaluation by pediatric ophthalmology. RESULTS: A total of 18/184 tested children had two variants in Usher syndrome-associated genes, including MYO7A, GPR98 (ADGRV1), USH2A, and PDZD7. SNHL varied from moderate to profound. 29% of the children who underwent clinical ophthalmology evaluation were found to have previously unidentified retinal abnormalities on retinal imaging or electroretinography consistent with inherited retinal degeneration. CONCLUSION: Among this ethnically and racially diverse pediatric population with apparently non-syndromic SNHL, HLGPT yielded a high proportion (10%) of children with two variants in genes associated with Usher syndrome. Early genetic testing allows early identification of variants conferring a diagnosis of Usher syndrome at a stage prior to visual symptoms. This allows for more informed genetic counseling, reproductive planning, and sensory deficit interventions. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:E2053-E2059, 2021.