An audiological evaluation of syndromic and non-syndromic craniosynostosis in pre-school going children

Goh, Liang Chye; Azman, Ali; Siti, Hufaidah binti Konting; Khoo, Wee Vien; Premala, a; Muthukumarasamy, Premala; Thong, Meow‐Keong; Bakar, Zulkiflee Abu; Manuel, Anura Michelle

doi:10.1016/j.ijporl.2018.03.010

Cited by 5 publications

(5 citation statements)

References 17 publications

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“…Our findings instead suggest sex differences in ophthalmologic and audiologic comorbidities, as NSC males were less likely to present with ophthalmological diagnoses or hearing loss than NSC females across all age groups. Early diagnosis and management of both of these conditions are essential to minimizing the risk for permanent damage [7][8][9][10]19]. Therefore, we recommend close monitoring of NSC patients to ensure any yet unreported sequelae associated with NSC are promptly managed.…”

Section: Discussionmentioning

confidence: 99%

“…The abnormal skull growth associated with craniosynostosis may impede development of the middle ear, increasing the risk of audiologic deficits such as chronic otitis media and hearing loss [17]. Though these outcomes are more commonly associated with syndromic cases [17, 19, 42, 43], some studies have explored the presence of chronic otitis media and hearing loss in the NSC population. Grewal et al [18] reported that 36% of their 113-patient NSC cohort had experienced at least one episode of otitis media, falling within the upper limit of normal when compared to historical rates among normocephalic children, and only 9% of NSC patients were found to have hearing loss; however, this study did not report a breakdown of prevalence rates by sex.…”

Section: Discussionmentioning

confidence: 99%

“…With these structural impacts on nearby anatomical features and the subsequent risk of increased ICP, craniosynostosis patients may present with consequent ophthalmological diagnoses, such as strabismus, papilledema, or exophthalmos [6][7][8][9][10]. Associations have also been reported with developmental delays, obstructive sleep apnea, chronic otitis media, hearing loss, chronic headaches, and seizure disorders [11][12][13][14][15][16][17][18][19][20][21]. Despite these correlations, substantial evolution in NSC management has led to improved outcomes in these patients, and the genetic pathophysiology of NSC craniosynostosis is becoming increasingly better understood with the development of next-generation sequencing technologies [22,23].…”

Section: Introductionmentioning

confidence: 99%

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Sex Differences in Comorbidities of Pediatric Craniosynostosis at Presentation

Presto¹,

Collins²,

Garza³

et al. 2022

Pediatr Neurosurg

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Introduction: Craniosynostosis is a common pediatric presentation in which the premature fusion of one or more cranial sutures results in a misshapen skull. This birth defect is often associated with comorbidities due to structural impacts on nearby anatomical features. While there is some evidence for a male-predominance among craniosynostosis patients, little has been investigated regarding sex differences in comorbidities of this condition. This study seeks to explore potential sexual dimorphisms in craniosynostosis patients at the time of presentation. Methods: We conducted a retrospective, cross-sectional review of male and female non-syndromic craniosynostosis (NSC) patients between the ages of 1 month and 9 years that were evaluated at a 500-bed academic hospital or a 977-bed private hospital in Lubbock, Texas, United States. Common comorbidities including ophthalmologic diagnoses, developmental delays, obstructive sleep apnea, chronic otitis media, hearing loss, chronic headaches, and seizure disorders were evaluated. The NSC cohort was compared to a similarly-aged trauma group that represented the normal population. Results: 175 NSC patients fit the inclusion criteria, of which 109 (62%) were male. A diagnosis of craniosynostosis was significantly associated with ophthalmological diagnoses (p < 0.0001), chronic otitis media (p < 0.0001), developmental delays (p < 0.0001), and hearing loss (p = 0.0047). Male NSC patients were less likely to present with ophthalmological diagnoses (p = 0.0010) or hearing loss (p = 0.0052) than females. Conclusions: Our findings expand on current literature evaluating possible comorbidities of NSC, particularly supporting the association with ophthalmological diagnoses, chronic otitis media, developmental delays, and hearing loss. We also report sex differences in ophthalmological diagnoses and hearing loss for NSC patients. These findings can serve to educate physicians of symptoms requiring prompt recognition and management in these patients.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Sex Differences in Comorbidities of Pediatric Craniosynostosis at Presentation

Presto¹,

Collins²,

Garza³

et al. 2022

Pediatr Neurosurg

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“…Middle ear cavitation and ossicles (Church et al, 2007;Farlie et al, 2016;Goh et al, 2018) Mandibulofacial dysostosis with alopecia (MFDA)…”

Section: Tbx1mentioning

confidence: 99%

Neural crest contributions to the ear: Implications for congenital hearing disorders

Martin

2019

Hearing Research

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Congenital hearing disorders affect millions of children worldwide and can significantly impact acquisition of speech and language. Efforts to identify the developmental genetic etiologies of conductive and sensorineural hearing losses have revealed critical roles for cranial neural crest cells (NCCs) in ear development. Cranial NCCs contribute to all portions of the ear, and defects in neural crest development can lead to neurocristopathies associated with profound hearing loss. The molecular mechanisms governing the development of neural crest derivatives within the ear are partially understood, but many questions remain. In this review, we describe recent advancements in determining neural crest contributions to the ear, how they inform our understanding of neurocristopathies, and highlight new avenues for further research using bioinformatic approaches.

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“…14,18 Moreover, hearing loss has been noted in both SCS and NSCS, which substantially impairs language acquisition and development. 12,[19][20][21] Hearing loss is markedly more common among patients with SCS, being as high as 92%, 80%, and 74% in Pfeiffer, Apert, and Crouzon syndrome respectively. 19 Several studies have shown improvements of language and speech after craniofacial surgical correction.…”

Section: Introductionmentioning

confidence: 99%

Are Patients With Syndromic Craniosynostosis at Greater Risk for Abnormal Speech and Language Development Than Patients With Non-syndromic Craniosynostosis?

Stanbouly,

Goudarzi,

Grillo

et al. 2023

FACE

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Objective: The aim of this study was to determine whether patients with syndromic craniosynostosis (SCS) are at increased risk for abnormal speech and language development (ASLD) relative to patients with non-syndromic craniosynostosis (NSCS). Study Design: A retrospective cohort study was completed using the Kids’ Inpatient Database (KID). All patients with craniosynostosis (CS) were included were included. The primary predictor variable was study grouping (SCS vs NSCS). The primary outcome variable was ASLD. Multivariate logistic regression were performed to identify risk factors for ASLD. Results: The final study sample included a total of 10 089 patients with craniosynostosis (CS) (37.7% female, 51.6% White, mean age 1.78 years). Patients with SCS were at increased risk for ASLD relative to patients with NSCS (OR 2.1, P < .001). After controlling for all other variables, patients with SCS were no longer at increased risk for ASLD relative to patients with NSCS (OR 1.2, P = .442). Conclusions: Relative to NSCS, SCS per se is not a risk factor for ASLD. The significantly greater prevalence of asthma, intracranial hypertension, and compression of brain, all of which are risk factors for ASLD, among patients with SCS explains the greater prevalence of ASLD in SCS relative to NSCS.

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An audiological evaluation of syndromic and non-syndromic craniosynostosis in pre-school going children

Cited by 5 publications

References 17 publications

Sex Differences in Comorbidities of Pediatric Craniosynostosis at Presentation

Sex Differences in Comorbidities of Pediatric Craniosynostosis at Presentation

Neural crest contributions to the ear: Implications for congenital hearing disorders

Are Patients With Syndromic Craniosynostosis at Greater Risk for Abnormal Speech and Language Development Than Patients With Non-syndromic Craniosynostosis?

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