Hearing Loss in Pediatric Patients With Cerebral Palsy.

OBJECTIVE: This study evaluates the prevalence, type, and severity of hearing impairment in children with cerebral palsy (CP) and to analyze audiologic and otologic outcomes in these patients. STUDY DESIGN: Retrospective analysis of the AudGen Database. SETTING: Tertiary academic referral center. PATIENTS: Pediatric patients in AudGen Database with a diagnosis of cerebral palsy. Appropriate audiologic, otologic, and demographic data were recorded.Nine hundred forty patients met inclusion criteria. Hearing loss (HL) was defined as greater than 15 dB HL at any threshold by pure tone or greater than 20 dB HL by soundfield audiometry. Other available otologic and medical conditions were documented. RESULTS: Of 940 patients, 367 (39%) had hearing loss. Of the 1,629 individual ears with HL, 782 (48%) had conductive, 63 (4%) had sensorineural, 410 (25%) had mixed, and 374 (23%) had unspecified hearing loss. Patients with mixed type 1 hearing loss had significantly worse PTAs. INTERPRETATION: Hearing loss in CP has a large degree of sensorineural loss, with a predisposition to be bilateral. The severity of hearing loss was correlated with the degree of the motor and neurologic disability in these patients.

Characteristics and Progression of Hearing Loss in Children with Down Syndrome.

OBJECTIVES: To evaluate hearing impairment in children with Down syndrome, and to describe the factors that influence the severity of hearing loss or changes in hearing over time. STUDY DESIGN: Using the Audiological and Genetic Database (AudGenDB), audiograms of children with Down syndrome were analyzed retrospectively for type, severity, and laterality of hearing loss, as well as changes over time. Medical charts and imaging results were reviewed to identify factors influencing hearing loss. RESULTS: Among the 1088 patients with Down syndrome included in the study, 921 had hearing loss in at least 1 ear, 91.1% had bilateral hearing loss, and 8.9% had unilateral hearing loss (1760 total ears with hearing loss). Of the ears with hearing loss, 18.8% (n = 180) had moderate or worse hearing loss. "Undefined" hearing loss and pure conductive hearing loss (CHL) were the most common types, followed by mixed hearing loss and sensorineural hearing loss (SNHL). Three-quarters (75.4%) of the children had experienced chronic otitis media or more than 2 episodes of acute otitis media. Patients with bilateral, mixed hearing loss or a history of seizures were at risk for more severe hearing loss. CHL, absence of cholesteatoma, and placement of first ear tubes before age 2 years were associated with greater improvement in hearing over time, whereas SNHL and mixed hearing loss were associated with progressive decline. CONCLUSION: Children with Down syndrome who have bilateral, mixed hearing loss or a history of seizures are at risk for more severe hearing loss. SNHL and mixed hearing loss should not be overlooked in patients with CHL. All patients with Down syndrome will benefit from serial audiograms, especially those children with SNHL or mixed hearing loss, which is likely to worsen over time.

Hearing loss in children with growth hormone deficiency.

OBJECTIVE: Although insulin-like growth factor 1 (IGF-1) has been shown to be important for inner-ear development in animal models, little is known about the otologic and audiologic findings of children with growth hormone deficiency (GHD). The goal of this study is to evaluate the prevalence, type, and severity of hearing impairment in children with GHD. METHODS: Audiologic, otologic, and demographic data were recorded for children with a diagnosis of GHD in the AudGen database. Data for each patient were selected based on the first encounter with available complete audiometric data or the first encounter with a type of hearing loss documented. The patients were then stratified by type and severity of hearing loss, and otologic issues were documented. A separate cohort comprised of children with GHD without hearing loss was compared as a control. RESULTS: 209 children with GHD met inclusion criteria. 173 (83%) of these patients had hearing loss. 79% of losses were bilateral and 21% were unilateral (309 total ears with hearing loss). 293 of the 309 ears with hearing loss had audiograms with ear-specific thresholds; 47 had conductive, 24 had sensorineural, 65 had mixed and 157 had undefined hearing loss with incomplete audiograms. Pure-tone averages (PTA) were higher among patients with mixed hearing loss compared to patients with all other loss types. CONCLUSION: Hearing loss is prevalent in children with GHD with a predisposition to be bilateral. These findings suggest the need for increased awareness and routine hearing screening for patients with GHD. Further studies may elucidate the etiology of the hearing impairment in children with GHD to better aid pediatricians, endocrinologists, otolaryngologists and audiologists when assessing and managing these children.

The influence of obesity on operating room time and perioperative complications in cochlear implantation.

OBJECTIVE: The rising incidence of obesity in the United States is associated with increased healthcare expenditures and resource allocation. Obesity has been associated with prolonged operating times during surgical procedures. The primary objective of this study is to compare body mass index (BMI) to length of surgery during cochlear implantation. METHODS: A retrospective case control study from a tertiary academic referral center was performed. Patients included were adults who underwent primary, single-sided cochlear implantation with documented BMI and operating room (OR) times from January 2009 to July 2015. The following data were collected: BMI, total operating room time (TORT), surgical operating room time (SORT), ASA status, perioperative and postoperative complications, age, and gender. RESULTS: Two hundreds and thirty-four patients were included and stratified into obese (BMI >30) and non-obese (BMI < 30) categories. Statistical analysis was performed comparing TORT against the obesity category along with other variables. Independent sample t-test demonstrated that obesity increases TORT and SORT by 16.8 min (P = 0.0002) and 9.3 min (P = 0.03), respectively, compared to the non-obese group. Multivariate linear regression analysis demonstrated no statistically significant impact of gender, or ASA status on total operating or surgical time. Obesity was associated with increased perioperative complications (odds ratio [OR], 6.21; 95% CI, 1.18-32.80; P = 0.03) and postoperative complications (OR, 3.97; 95% CI, 1.29-12.26; P = 0.02). CONCLUSIONS: Obesity leads to longer TORT and SORT during primary cochlear implant surgery. Obesity is also associated with increased perioperative and postoperative complications compared to non-obese patients. These data have implications with utilization of operating room resources.

Otologic and Audiologic Outcomes in Pediatric Patients With Velo-Cardio-Facial (22q11 Deletion) Syndrome.

OBJECTIVE: The focus of this study was to evaluate the prevalence, type, and severity of hearing impairment in patients with velo-cardio-facial syndrome (VCFS) and to compare these characteristics with patient demographics and other otologic factors. STUDY DESIGN: Retrospective analysis of the AudGen Database. SETTING: Tertiary academic referral center. PATIENTS: Pediatric patients in AudGenDB with a diagnosis of velo-cardio-facial syndrome or DiGeorge syndrome. INTERVENTIONS: Appropriate audiologic, otologic, and demographic data were recorded. MAIN OUTCOME MEASURE: Four hundred six patients met inclusion criteria. Data for each patient were selected based on their encounters with earliest complete audiometric data, and hearing loss (HL) was defined as greater than 15 dB HL at any threshold by pure tone or greater than 20 dB HL by soundfield audiometry. The patients were then stratified by type and severity of HL, and available otologic and medical conditions were documented. RESULTS: Two hundred forty eight (60.7%) patients had HL. Of the 391 individual HL ears, 127 had conductive, 22 had sensorineural, 115 had mixed, and 127 had unspecified hearing loss. 57% of the loss was bilateral. Patients with mixed HL had significantly worse pure-tone averages (PTAs). CONCLUSION: HL is prevalent in patients with VCFS. The hearing loss is primarily of conductive origin, with a predisposition to be bilateral, with mixed HL typically have more severe loss. There were a large number of patients with sensorineural hearing loss components as well. Further studies are needed to investigate the causal nature of the hearing impairment in VCFS, to better aid otolaryngologist and audiologists when assessing these patients.

Audiologic and otologic phenotype in children with Duane's Retraction Syndrome: A rare ophthalmologic disorder.

OBJECTIVE: The focus of this study was to evaluate the prevalence, type, and severity of hearing impairment in patients with Duane's Retraction Syndrome and to relate these measures to patient demographics and other otologic and audiologic factors. STUDY DESIGN: Retrospective AudGen Database analysis SETTING: Tertiary academic referral center PATIENTS: Pediatric patients in AudGenDB with a diagnosis of Duane's Retraction Syndrome (DRS). INTERVENTIONS: Appropriate audiologic, otologic, and demographic data were recorded. MAIN OUTCOME MEASURE: Seventy-nine patients (n = 79) met inclusion criteria. The first encounter with available audiometric data or the first encounter with hearing loss were documented. Audiograms were stratified by type and severity of hearing loss, and common associated medical issues were documented. RESULTS: 57 children had normal hearing; 22 had hearing loss. 9 ears had pure conductive hearing loss, 1 had pure sensorineural, 14 ears had components of both, and 79 had hearing loss that could not be specified. Multivariate regression revealed episodes of chronic otitis and craniofacial anomalies are associated with worse hearing loss. CONCLUSION: This study presents a detailed characterization of hearing loss in patients with Duane's retraction syndrome. Conductive and sensorineural hearing loss are both prevalent among these children. Careful and early audiologic evaluation of all patients with DRS is important.

Audiologic Outcomes in Ehlers-Danlos Syndrome.

OBJECTIVE: The focus of this study was to evaluate the prevalence, type, and severity of hearing impairment in patients with Ehlers-Danlos Syndrome (EDS) and to compare these characteristics with patient demographics and other otologic factors. STUDY DESIGN: Retrospective analysis of the AudGen Database. SETTING: Tertiary academic referral center. PATIENTS: Pediatric patients in AudGenDB with a diagnosis EDS. INTERVENTIONS: Appropriate audiologic, otologic, and demographic data were recorded. MAIN OUTCOME MEASURE: One hundred forty one patients met inclusion criteria. Data for each patient were selected based on their first encounter by age with available audiometric data or their first encounter with a type of hearing loss documented. The patients were then stratified by type and severity of hearing loss, and available otologic issues were documented. RESULTS: One hundred nine patients had normal hearing. Thirty two patients had hearing loss, with 19 bilateral, and 13 unilateral. Of the 51 individual ears, 25 had conductive, 23 had sensorineural, and 3 had mixed hearing loss. Audiometric configuration was primarily flat in nature. The hearing loss severity was significantly correlated with age, but not influenced by the type of loss. CONCLUSION: Hearing loss is prevalent in patients with EDS. The hearing loss is equally of conductive and sensorineural origin, with a predisposition to be bilateral. Further evaluation of the underlying pathology of hearing loss in these patients is warranted to aid otolaryngologists and audiologists in diagnosis and management.