Wideband acoustic immittance in children with Down syndrome: prediction of middle-ear dysfunction, conductive hearing loss and patent PE tubes.

OBJECTIVE: The purpose of this study was to evaluate pressurised wideband acoustic immittance (WAI) tests in children with Down syndrome (DS) and in typically developing children (TD) for prediction of conductive hearing loss (CHL) and patency of pressure equalising tubes (PETs). DESIGN: Audiologic diagnosis was determined by audiometry in combination with distortion-product otoacoustic emissions, 0.226 kHz tympanometry and otoscopy. WAI results were compared for ears within diagnostic categories (Normal, CHL and PET) and between groups (TD and DS). STUDY SAMPLE: Children with DS (n = 40; mean age 6.4 years), and TD children (n = 48; mean age 5.1 years) were included. RESULTS: Wideband absorbance was significantly lower at 1-4 kHz in ears with CHL compared to NH for both TD and DS groups. In ears with patent PETs, wideband absorbance and group delay (GD) were larger than in ears without PETs between 0.25 and 1.5 kHz. Wideband absorbance tests were performed similarly for prediction of CHL and patent PETs in TD and DS groups. CONCLUSIONS: Wideband absorbance and GD revealed specific patterns in both TD children and those with DS that can assist in detection of the presence of significant CHL, assess the patency of PETs, and provide frequency-specific information in the audiometric range.

Hearing loss in Down Syndrome revisited - 15 years later.

OBJECTIVE: In 2001, the senior authors published a study investigating hearing loss in young children (ages 11 months to 3.8 years) with Down Syndrome (DS). We re-visit this same study population to review current audiologic status, the incidence of pressure equalization tube (PET) placement(s), and rate of tympanic membrane (TM) perforations. We aim to better understand the natural history of ear disease and hearing loss in DS and assess potential complications. METHODS: This retrospective chart review included 57 children with DS who previously completed in 2006, a 5 year, longitudinal study investigating otolaryngologic problems in DS. Updated audiologic data was available for 54. Audiograms, age of ear specific testing, PET placement(s), and tympanic membrane(TM) descriptions were reviewed. RESULTS: Ages ranged from 14 to 18 years (mean 16.34 years). PET placement occurred in 88.8%, with mean of 3.5 procedures. 30% of PET's were placed after age 6. Ear specific testing was obtained in 92.5% (mean age 4.54 years). Normal hearing was present in 44% (right ear) and 38% (left ear). "Functional" hearing levels, defined as normal or mild hearing loss and speech reception threshold ≤ 30 dB, occurred in 83.3%. Sensorineural/mixed hearing loss was present in 11% (right ear) and 9% (left ear). TM perforations rate was 17%. No cholesteatomas were found. CONCLUSION: Chronic otitis media and indications for PET's persist as children with DS age. Although functional hearing occurred in 83.3%, there was an overall decrease in hearing levels as the children aged. Tympanic membrane perforations occurred in 17%. Continued surveillance of otologic and audiologic status in patients with Down syndrome is recommended.

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.

Guided growth for the treatment of limb length discrepancy: a comparative study of the three most commonly used surgical techniques.

The purpose of this study was to compare the safety and effectiveness of three mechanical devices (percutaneous transphyseal screws, tension band plates, and staples) for the correction of limb length discrepancies in growing children and adolescents. Thirty-nine consecutive patients treated with epiphysiodesis for limb length discrepancy were retrospectively reviewed. No significant difference was recorded between the three devices in postoperative limb length discrepancy or the rate of correction between the plating and stapling groups and between plating and percutaneous transphyseal screws groups. Epiphysiodesis for length discrepancies can be performed effectively using staples, tension band plates, or percutaneous transphyseal screws.