Effects of adeno-tonsillectomy on polysomnography patterns in Down syndrome children with obstructive sleep apnea: A comparative study with children without Down syndrome

Objective

To determine if adeno-tonsillectomy (T&A) in children with Down syndrome (DS) improves breathing, measured by apnea hypopnea index (AHI), rapid eye movement AHI (REM-AHI) and the lowest oxygen desaturation (SaO₂), and sleep disruption, measured by arousal index (ArI) and time spent in stages 1-4 and rapid eye movement (REM) sleep and compare these results with a group of non-DS children with obstructive sleep apnea (OSA).

Study design

Retrospective chart review at pediatric sleep center.

Patients

Eleven DS and nine non-DS children underwent pre- and post-T&A polysomnography between 1997 and 2005.

Outcome measures

Pre- and post-T&A polysomnography parameters were compared using paired t-test and independent samples test.

Results

Mean age in DS group was 101 months and non-DS group was 80 months (64% males in DS and 88% in non-DS group). The average BMI was 29.8 and 27.6 for DS and non-DS group. The total AHI showed significant improvement after T&A but this was not as marked as the non-DS group. REM-AHI and lowest SaO₂ did not show significant change in the DS children. The non-DS group showed significant improvement in all respiratory parameters. Both groups showed mild improvement in sleep parameters. With the modest overall improvement, 27% of the DS children required no further treatment. However, 73% required CPAP, BiPAP or oxygen for persistent OSA.

Conclusion

This study supports the fact that T&A in DS children improves some parameters of OSA, however not as markedly as in non-DS children.     

Wideband acoustic absorbance in children with Down syndrome

Introduction

Tympanometry is currently the most frequently used tool for assessing the status of the middle ear, commonly assessed using a single 226 Hz tone. However, the use of the Acoustic Immittance Measures with a wideband stimulus is a promising high-resolution evaluation, especially in individuals known to have middle ear alterations, such as Down syndrome patients.

A comparison of tympanometry with 226 Hz and 1000 Hz probe tones in children with Down syndrome

Background

For children with Down syndrome, the incidence of hearing loss may be as high as 78% [1], therefore the American Academy of Pediatrics recommends regular screening for the presence of hearing abnormalities. Tympanometry is used as an indication of middle ear pathology. In our experience, Down's patients’ tympanograms do not always correlate with otoscopic findings. Down's patients have joint laxity, small ear canals, anterior tympanic membrane orientation and softer tissue composition, all factors thought to affect tympanogram results in infants. Because the use of the 1000 Hz tympanometry is widely recognized as standard procedure in the evaluation of infants aged 0–6 months, we propose it may have greater reliability in testing patients with Down syndrome.

Objective

Compare the results of visual inspection of the tympanic membrane by a Pediatric Otolaryngologist to the results of tympanometry at traditional probe tone (226 Hz) and at the infant probe tone (1000 Hz).

Methods

Institutional Review Board – approved prospective study of 26 subject-ears in patients with Down syndrome aged 6 months–18 years but recent stable middle ear/Eustachian tube function using physical examination and tympanometric probe tones at 226 Hz and 1000 Hz.Subject-ears were examined with record of “clear of effusion,” showed the presence of “fluid,” or were to be “excluded.” Blinded to ear exam results, tympanometry was then completed with record of which Jerger classification tympanogram was found at each frequency.

Results

Although the sensitivity of each test was 1, the specificity of the 1000 Hz tympanometry (100%) in this study was markedly improved compared to the specificity of the 226 Hz tympanometry (71%) (p = 0.016).

Conclusions

This pilot study demonstrated evidence that tympanometry in children with Down syndrome may be more reliable at 1000 Hz than at 226 Hz in detecting the presence of middle ear effusion beyond infancy. Use of the 1000 Hz probe tone yielded fewer false positives for disease (type B tympanograms in the setting of absent middle ear disease). Further studies of a larger patient population are needed to corroborate these results.     

The management of children with Down syndrome and profound hearing loss

Introduction: Although, the association between Down syndrome (DS) and conductive hearing loss is well recognized, the fact that a small proportion of these children may have a severe to profound sensorineural hearing loss that could benefit from cochlear implantation (CI) is less well understood. The management of significant co-morbidities in children with DS can delay initial diagnosis of hearing impairment and assessment of suitability for CI can likewise be challenging, due to difficulties conditioning to behavioural hearing tests.

Methods: We performed a retrospective case note review of three children with DS referred to the Manchester Cochlear Implant Programme.

Results: Three illustrative cases are described including CI in a 4 years old. Using conventional outcome measurement instruments, the outcome could be considered to be suboptimal with a Categories of Auditory Performance score of 4 at 6 months post-op and at last follow up. In part, this is likely to reflect the delay in implantation, but the role of cognitive impairment must be considered. The cases described emphasize the importance of comprehensive radiological and audiological assessment in children with DS being considered for CI.

Conclusion: The influence of cognitive impairment upon outcome of CI must be taken into account, but should not be considered a contra-indication to implantation in children with DS. Benefit that might be considered limited when quantified using existing general outcome measurement instruments, may have a significant impact upon psychosocial development and quality of life in children with significant cognitive impairment, or other additional needs.     

Sleep-disordered breathing in children with Down syndrome: Usefulness of home polysomnography

ObjectiveTo investigate the technical feasibility of unattended home polysomnography (HPSG) in children with Down syndrome.MethodsData from children with Down syndrome under 10 years of age referred to a diagnostic sleep study was analyzed. A full sleep-lab based polysomnography (PSG) or a HPSG with a portable device was performed. Uninterpretable HPSGs were defined as: recordings with (i) loss of ≥2 of the following channels: nasal flow, or thoracoabdominal sensors, or (ii) HPSG with less than 4 h of artifact-free recording time or (iii) less than 4 h SpO₂ (peripheral capillary oxygen saturation) signal.ResultsA total of 44 children (68% males) were included in the study, with a mean age of 3.6 (0.1–10) years. PSG was performed in 8 cases and HPSG in 36 cases. Six HPSG recordings were classified as uninterpretable and had to be repeated. Age, gender and BMI were no significant predictors of uninterpretability of the HPSG. Obstructive sleep apnea (OSA) was present in 61% (n = 27) of all subjects, and classified as mild, moderate, and severe in 43% (n = 19), 11% (n = 5), and 7% (n = 3) of cases, respectively. Interpretable and technically acceptable HPSGs were obtained in 30 subjects (83%). Age, gender and BMI were no significant predictors for interpretability of the HPSG.DiscussionThis study demonstrates that a portable polysomnographic home device may be helpful for diagnosing OSA in children with Down syndrome. Considering the potential consequences of untreated OSA, this screening test may be helpful for early diagnosis of OSA in children with Down syndrome.     

学龄唐氏综合征患儿与正常儿童口腔共鸣声学特征比较

目的探讨7～16岁唐氏综合征儿童与正常儿童口腔共鸣声学特征的差异。方法29例7～16岁唐氏综合征儿童和90例7～16岁正常儿童发/a/、/u/、/i/音各3次,每次1～2秒,采用启音博士言语测量仪分别测量两组3个元音的第一共振峰（F1）、第二共振峰（F2）、舌距和下颌距,比较两组结果。结果随年龄增长,正常儿童的F2（a ）呈显著降低的趋势、F2（i ）和舌距则呈显著增高的趋势,而唐氏综合征患儿的F2（i ）呈显著增高趋势;不同性别正常儿童各口腔共鸣指标均无显著差异,而唐氏综合征男童的 F1（i ）和 F1（u ）显著低于唐氏综合征女童（P＜0．05）,其下颌距则显著大于唐氏综合征女童（P＜0．05）;唐氏综合征患儿的F2（u）和F1（i）显著大于正常儿童（分别为 P＜0．001和 P＜0．01）,而舌距显著小于正常儿童（ P＜0．001）。结论随着年龄的增长,正常儿童舌向前运动的幅度和舌前后运动的范围会显著增加,口腔聚焦点会逐渐前移,而唐氏综合征患儿舌的向后运动幅度及前后运动范围的发展均落后于正常儿童,更易出现口腔共鸣障碍。     

Ear,nose and throat disorders in children with Down syndrome

OBJECTIVE: To document the reasons for which children with Down syndrome were referred to a pediatric otolaryngology practice, the underlying causes for these referrals, and the complications of routine surgical therapy. STUDY DESIGN: The study is a retrospective review of children referred to the Pediatric Otolaryngology Clinic at the University of New Mexico Health Sciences Center (Albuquerque, NM) during a period of 2.5 years. METHODS: Data were collected on 55 parameters related to ethnicity, demographics, diagnosis, surgical therapy, complications, and systemic comorbid conditions. RESULTS: The ethnicity of the study population was predominantly Hispanic or Latino (62%). The majority of children (76%) were referred for upper airway obstruction. Obstructive sleep apnea and laryngomalacia were the most common disorders in these children. An otological disorder was diagnosed in 70% of the children. Complications occurred after 27% of procedures for insertion of pressure equalization (PE) tubes to treat recurrent otitis media. Systemic comorbid conditions were present in 93% of the children, and the most common was gastroesophageal reflux disease. CONCLUSIONS: Obstructive sleep apnea and laryngomalacia were the most common reasons for referral of children with Down syndrome. Routine surgical procedures that required general anesthesia caused complications that are not common in other children. Treatment for systemic comorbid conditions should be considered as a component of therapy for otolaryngological disorders in children with Down syndrome.     

A rare case of co-existing Treacher-Collins and Down syndrome

Both Treacher-Collins (TCS) and Down syndrome cause structural and functional airway abnormalities. We present the case of a neonate with both syndromes who required tracheostomy in the first hour of life due to severe upper airway obstruction. The co-occurrence of both syndromes is rare, and presents unique challenges in securing the airway in the perinatal period.     

Hearing loss in Chinese school children with Down syndrome

OBJECTIVE: There is well-documented evidence in the literature concerning a high prevalence of deafness in children with Down syndrome (DS). The aim of this study was to examine the extent of hearing impairment and address the rehabilitation needs of a Chinese population with DS who were either in special schools or integrated into mainstream schools. METHODS: This study screened 92 children with DS at their own schools and 11 were reassessed in the University of Hong Kong Hearing Centre. Hearing status of the children with DS was analyzed on the basis of their screening and reassessment results for tympanometric, transient evoked otoacoustic emission (TEOAE) and pure tone audiometric examinations. RESULTS: A high point prevalence of hearing impairment (78% by ears or 90% by participants) in a Chinese school-aged sample of children with DS was noted. The most common degree of loss was mild to moderate. No significant gender difference, age effect or ear asymmetry was found for tympanometric failure or absence of TEOAE. An unfavorable mean speech intelligibility index score (0.2) was found for this group of children. CONCLUSIONS: Sound field amplification and suitable acoustic modifications to classrooms were recommended for Chinese children with DS in Hong Kong to improve their listening and learning environment. The point prevalence of hearing impairment in older children with DS in this study was in contrast to a previous local study on a younger age group. Further effort is needed to determine the role of possible aging effects on the type and prevalence of hearing impairment in populations with DS.     

The effect of adenotonsillectomy on obstructive sleep apnea in children with Down syndrome*

Objective: Children with Down syndrome (DS) are liable to develop obstructive sleep apnea (OSA) due to many anatomical airway abnormalities. The tonsils and adenoid occupy part of the airway space, and their removal may be helpful in relieving airway obstruction. The aim of this study was to assess the effectiveness of adenotonsillectomy in the treatment of OSA in those children.

Methods: Fifty DS children with difficult breathing were recruited, and they were subjected to polysomnographic examination (PSG). Patients with apnea-hypopnea index (AHI)?>?1 were considered to have OSA. Adenotonsillectomy was performed for patients who had OSA and adenotonsillar hypertrophy, and after 3 months PSG was done for them with recording of the same preoperative parameters.

Results: Forty-three children demonstrated OSA on PSG, and they were included in the study. The preoperative mean AHI was 9.18 (±?6.17) that improved postoperatively to 2.72 (±?3.80) with its normalization in 72% of patients. Also, significant improvement of arousal index, minimum oxygen saturation, desaturation index, and peak end-tidal CO₂ was achieved postoperatively.

Conclusion: Adenotonsillectomy is an effective method for the treatment of OSA in children with DS. However, the condition may persist in some children who usually have airway narrowing at multiple levels.     

The role of bone anchored hearing aids in children with Down syndrome

OBJECTIVES: To evaluate complication rates and outcomes of children with Down syndrome fitted with a Bone Anchored Hearing Aid (Baha). To evaluate whether the Bone Anchored Hearing Aid is a successful form of aural rehabilitation in children with Down syndrome from a patients' perspective. STUDY DESIGN: Retrospective case analysis and postal questionnaire study. SETTING: The Birmingham Children's Hospital, UK. METHODS: A total of 15 children were fitted with a Baha between February 1992 and February 2007. The age range was 2-15 years. A postal questionnaire was sent to each family. The Glasgow Children's Benefit Inventory (GCBI) was used in this study. OUTCOME MEASURES: Implantation results, skin reactions and other complications were recorded. Quality of life after receiving a Baha was assessed with the GCBI. RESULTS: All 15 patients are using their Baha 7 days a week for more than 8h a day after a follow-up of 14 months with continuing audiological benefit. No fixtures were lost, and skin problems were encountered in 3 (20%). Regarding quality of life, all 15 patients had improved social and physical functioning as a result of better hearing. CONCLUSIONS: Baha has an important role in the overall management of individuals with Down syndrome after conventional hearing aids and/or ventilation tubes have been considered or already failed. This study has shown a 20% rate of soft tissue reaction and there were no fixture losses in this group. No significant increase in complication rates was identified in children with Down syndrome. Finally, there was a significantly improved quality of life in children with Down syndrome after receiving their Baha. There was a high patient/carer satisfaction with Baha. Two of our series had bilateral two stage fixture procedures without any complications. More consideration should be given to bilateral bone anchored hearing aids in this group.     

Outcomes of tympanostomy tube placement in children with Down syndrome—A retrospective review

Objectives

Tympanostomy tubes are commonly used for treatment of chronic otitis media with effusion (COME) or recurrent acute otitis media (RAOM) in patients with Down syndrome, but hearing outcomes in this population have been mixed, and complications appear to be common. We aim to characterize outcomes and complications associated with tympanostomy tube placement in this population.

Methods

Retrospective review. All patients with Down syndrome presenting to a tertiary academic pediatric otolaryngology practice over a ten year period from 2002 to 2012 who received tympanostomy tubes for COME, RAOM, or hearing loss were reviewed.

Results

Long term follow up data was obtained in 102 patients, with average follow up 4.7 years. COME was the primary indication for tube placement in 100/102 (98%). Less than half of these patients (44%) initially failed their newborn hearing screen. Post operative hearing was found to be normal or near normal for the better hearing ear in 85/99 (85.9%), and normal to near normal in bilateral ears in 71/99 (71%). A majority (63.7%) of patients required two or more sets of tubes during the follow up period. Long term complications were common and were significantly increased if the patient required three or more sets of tubes, including chronic perforation (36.6% vs 8.2%, p < 0.001), atelectasis (29.3% vs 1.6%, p < 0.0001), and cholesteatoma (14.6% vs 0%, p = 0.003).

Conclusions

COME is a frequent problem in Down syndrome, and the majority of patients will require two or more sets of tubes during their childhood and achieve normal postoperative hearing. Long term complications of otitis media appear to be more common in this population and appear to correlate with increasing number of tubes placed. More investigation is required to determine optimal treatment strategies for COME in patients with Down syndrome.     

Inner ear dysplasia is common in children with Down syndrome (trisomy 21)

OBJECTIVES/HYPOTHESIS: Middle and external ear anomalies are well recognized in Down syndrome (DS, trisomy 21). Inner ear anomalies are much less frequently described. This study reviews inner ear morphology on imaging to determine the prevalence of cochlear and vestibular anomalies in children with DS. STUDY DESIGN: The authors conducted a retrospective review of imaging features of (DS) inner ear structures. METHODS: Fifty-nine sequential patients with DS with imaging of the inner ear were identified by a radiology report text search program. Quantitative biometric assessment of the inner ear was performed on patients with high-resolution computed tomography or magnetic resonance images of the petrous bone. Petrous imaging was performed for evaluation of inflammatory disease or hearing loss. Spinal imaging, which included petrous views, was performed in most cases to exclude C1 to 2 dislocation, a potential complication of DS. Measurements were compared with normative data. RESULTS: Inner ear dysplasia is much more common in DS than previously reported. Inner ear structures are universally hypoplastic. Vestibular malformations are particularly common and a small bony island of the lateral semicircular canal (<3 mm in diameter) appears highly typical. Additional findings in some patients were persistent lateral semicircular anlage with fusion of the lateral semicircular canal and vestibule into a single cavity, vestibular aqueduct and endolymphatic sac fossa enlargement, cochlear nerve canal hypoplasia, and stenosis or duplication of the internal auditory canal. Stenosis of the external meatus, poor mastoid pneumatization, middle ear and mastoid opacification, and cholesteatoma were common, as expected.     

Anatomic factors affecting the use of ultrasound to predict vocal fold motion: A pilot study

Purpose

Ultrasonography is a well-established modality for visualization of head and neck anatomy. Using ultrasound to detect vocal fold mobility has been described before, but no study has evaluated factors affecting the exam reliability. The aim of the study is to determine anatomic factors influencing the reliability of ultrasound to detect vocal fold motion.Methods and materialsPatients underwent ultrasound evaluation and flexible laryngoscopy to assess vocal fold motion from August 2015 to March 2016. Length, accuracy, and clarity of ultrasound examination were assessed, compared to flexible laryngoscopy. For patients with prior neck CT scan imaging, laryngeal anatomy was independently assessed by a blinded neuroradiologist.

Vocal tract dimensional development of adolescents: An acoustic reflection study

Objective

The purpose of the study was to investigate the effects of age and gender on adolescents’ vocal tract dimensional development with acoustic reflection technology (ART).

Methods

A total of ninety-five male and female adolescents aged between 10 and 18 divided into three age groups were tested with acoustic reflection technology (ART) and acoustic program to secure their vocal tract dimensional parameters and the vowel formant frequencies.

Results

Significant age and gender effects were found not only in vocal tract length, but also segmental volumetric measurements, as well as the vowel formant frequencies.

Conclusions

The findings of this study have provided insights on the developmental trend of adolescents’ vocal tracts. The study has also offered a preliminary anatomical database of adolescents’ vocal tract dimensional growth for otolaryngologists, clinical anatomists, speech therapists and other health professionals of swallowing, respiration and communicative disorders.     

Cine magnetic resonance imaging: evaluation of persistent airway obstruction after tonsil and adenoidectomy in children with Down syndrome

OBJECTIVE/HYPOTHESIS: Although usually successful, not all obstructive sleep apnea is cured by removal of the tonsils and adenoids (T&A). This is particularly true in children with Down syndrome and craniofacial anomalies. This is because of the multiple levels of obstruction in their airways, with obstruction present not only at the level of the tonsils and adenoids but also from base of tongue obstruction, soft palate collapse, and hypopharyngeal collapse. The cine magnetic resonance image (MRI) is useful in evaluating the upper airway in those patients who have not achieved a normal polysomnogram after T&A surgery. STUDY DESIGN: Prospective case series. METHODS: Fifteen children with Down syndrome who had previously undergone a T&A but continued to have abnormal postoperative polysomnograms underwent a cine MRI with fast gradient cine MRI images. RESULTS: The cine MRI identified different areas and levels of obstruction that ultimately affected the children's treatment courses. Recurrent adenoid tissue, glossoptosis, soft palate collapse, hypopharyngeal collapse, and enlarged lingual tonsils were identified. The results as well as several illustrative cases are presented. CONCLUSION: Cine MRI evaluates upper airway obstruction in children who may have multiple sources of obstruction causing their obstructive sleep apnea. This is particularly helpful in children with Down syndrome and craniofacial anomalies. This technology is useful in all children with complex upper airway obstruction.     

Safety of neck rotation for ear surgery in children with Down syndrome

OBJECTIVE: Seek information about spinal cord safety for children with Down syndrome positioned for ear surgery. STUDY DESIGN: Prospective consecutive patients, each serving as his or her own control. METHODS: Somatosensory evoked potentials were recorded from 17 children who were undergoing elective otolaryngological surgery. None of the patients had neurological symptoms or physical examination findings suggesting cervical spinal cord embarrassment. Specifically, muscle tone was normal or mildly reduced globally, consistent with Down syndrome, and deep tendon reflexes were normal and not appreciably different in the upper and lower extremities. On plain lateral radiographs obtained in the neutral, flexed, and extended positions, the patients' cervical spines were normal. RESULTS: When the anesthetized children had their necks placed in either right or left 60 degrees rotation, no significant change in somatosensory latency or amplitude was found. With more than 99.999% certainty, neurologically intact children with Down syndrome with normal plain cervical spine radiographs were not exposed to extra risks by 60 degrees neck rotation during surgery. CONCLUSION: Patients with Down syndrome who are neurologically intact and who have normal lateral neck radiographs do not appear at great risk with neck rotation.     

Quality of life improvement after pressure equalization tube placement in Down syndrome: A prospective study

ObjectiveTo evaluate quality-of-life changes after bilateral pressure equalization tube placement with or without adenoidectomy for the treatment of chronic otitis media with effusion or recurrent acute otitis media in a pediatric Down syndrome population compared to controls.Study designProspective case-control observational study.MethodsThe OM Outcome Survey (OMO-22) was administered to both patients with Down syndrome and controls before bilateral tube placement with or without adenoidectomy and at an average of 6–7 months postoperatively. Thirty-one patients with Down syndrome and 34 controls were recruited. Both pre-operative and post-operative between-group and within-group score comparisons were conducted for the Physical, Hearing/Balance, Speech, Emotional, and Social domains of the OMO-22.ResultsBoth groups experienced improvement of mean symptom scores post-operatively. Patients with Down syndrome reported significant post-operative improvement in mean Physical and Hearing domain item scores while control patients reported significant improvement in Physical, Hearing, and Emotional domain item scores. All four symptom scores in the Speech domain, both pre-operatively and post-operatively, were significantly worse for Down syndrome patients compared to controls (p ≤ 0.008).ConclusionsSurgical placement of pressure equalizing tubes results in significant quality of life improvements in patients with Down syndrome and controls. Problems related to speech and balance are reported at a higher rate and persist despite intervention in the Down syndrome population. It is possible that longer follow up periods and/or more sensitive tools are required to measure speech improvements in the Down syndrome population after pressure equalizing tube placement ± adenoidectomy.