Query: An overview of middle ear disease in cleft palate children.

title: An overview of middle ear disease in cleft palate children.

title: Eustachian Tube Function in Cleft Palate Children
abstract: The cleft palate population has a high prevalence of chronic otitis media with effusion (OME) The present study attempts to relate this pathology to a functional obstruction of the eustachian tube (ET) Employing two methods, the forced response test and the inflation deflation test, various parameters of ET function were evaluated in 41 children and adolescents with cleft palates. The results indicated that cleft palate children have a limited ability to open the ET actively by swallowing, as evidenced by an inability to equilibrate applied positive or negative pressures. These children demonstrated little ability to alter pressure in the middle ear (ME) by either the Valsalva or Toynbee maneuver. Passive ET airflow resistance in these children is not different from that of a traumatic perforation group. However, in the majority of cases (73% the resistance of the tube increased with swallowing, suggesting a constriction of the ET rather than a dilation. Further, for those individuals capable of tubal dilation, the efficiency of dilation as measured by the resistance ratio was significantly less than that reported for the traumatic perforation group. Eustachian tube function in this population was not demonstrably dependent on age, sex, or type of cleft. The cleft palate children of the present study had severe functional obstruction of the ET. This obstruction was primarily related to the inability of the tensor veli palatini (TVP) muscle to dilate the ET actively during swallowing and appears to be the major factor responsible for the pathogenesis of OME in this population.

title: Cleft lip and palate
abstract: Clefts of the lip and palate are generally divided into two groups, isolated cleft palate and cleft lip with or without cleft palate, representing a heterogeneous group of disorders affecting the lips and oral cavity. These defects arise in about 1.7 per 1000 liveborn babies, with ethnic and geographic variation. Effects on speech, hearing, appearance, and psychology can lead to longlasting adverse outcomes for health and social integration. Typically, children with these disorders need multidisciplinary care from birth to adulthood and have higher morbidity and mortality throughout life than do unaffected individuals. This Seminar describes embryological developmental processes, epidemiology, known environmental and genetic risk factors, and their interaction. Although access to care has increased in recent years, especially in developing countries, quality of care still varies substantially. Prevention is the ultimate objective for clefts of the lip and palate, and a prerequisite of this aim is to elucidate causes of the disorders. Technological advances and international collaborations have yielded some successes.

title: Abnormal brain structure in children with isolated clefts of the lip or palate.
abstract: To evaluate brain structure in a sample of children with isolated clefts of the lip and/or palate (ICLP) DESIGN Case control study.  Tertiary care center.  A large sample of 74 children aged 7 to 17 years with ICLP was compared with a healthy control group, matched by age and sex. MAIN EXPOSURE Isolated cleft lip and/or palate.  General measures of height and head circumference were obtained. Brain structure was evaluated using magnetic resonance imaging, generating both general and regional brain measures (volumes) RESULTS Height was significantly lower in the ICLP group (F 4.83, P .03) After controlling for this smaller body size, children with ICLP had abnormally small brains with both cerebrum (F 4.47, P .04) and cerebellum (F 14.56, P .001) volumes substantially decreased. Within the cerebrum, the frontal lobe was preferentially decreased (F 7.22, P .008) and subcortical nuclei were also substantially smaller (F 4.18, P .003) Tissue distribution of cortical gray matter and white matter within the cerebrum were abnormal in boys with ICLP (larger cortical volume, smaller volume of white matter) but proportional to controls in girls with ICLP.  Children with ICLP have abnormal brain structure, potentially due to abnormal brain development. The fact that the pattern of brain abnormalities in children with ICLP is dramatically different from the pattern of brain abnormalities seen in adults with ICLP suggests that brain growth and development trajectory is also abnormal in subjects with ICLP.

title: Gene/environment causes of cleft lip and/or palate
abstract: Craniofacial anomalies, and in particular cleft lip and palate, are major human birth defects with a worldwide frequency of 1 in 700 and substantial clinical impact. A wide range of studies in developmental biology has contributed to a better knowledge of how both genes and environmental exposures impact head organogenesis. Specific causes have now been identified for some forms of cleft lip and palate, and we are at the beginning of a time in which the common nonsyndromic forms may also have specific etiologies identified. Mouse models have an especially important role in disclosing cleft etiologies and providing models for environmental cotriggers or interventions. An overview of the gene environment contributions to nonsyndromic forms of clefting and their implications for developmental biology and clinical counseling is presented.

title: Cleft Palate and Craniofacial Anomalies: Effects on Speech and Resonance
abstract: Part I: Normal and Abnormal Craniofacial Features Chapter 1: Anatomy and Physiology: The Orofacial Structures and Velopharyngeal Valve Chapter 2: Genetics and Patterns of Inheritance Chapter 3: Clefts of the Lip and Palate Chapter 4: The Genetics Evaluation and Common Craniofacial Syndromes Part II: Problems Associated with Clefts and Craniofacial Anomalies Chapter 5: Feeding Problems of Infants with Cleft Lip/Palate or Craniofacial Anomalies Chapter 6: Developmental Concerns in Children with Clefts and Craniofacial Syndromes Chapter 7: Resonance Disorders and Velopharyngeal Dysfunction Chapter 8: Ear, Nose, and Throat Anomalies: Effects on Speech and Resonance Chapter 9: Dental Anomalies Associated with Cleft Lip and Palate Chapter 10: Psychosocial Aspects of Cleft Lip/Palate and Craniofacial Anomalies Part III: Interdisciplinary Care Chapter 11: The Team Approach to Assessment and Treatment Part IV: Assessment Procedures: Speech, Resonance, and Velopharyngeal Dysfunction Chapter 12: Assessment Using Low Tech and No Tech Procedures Chapter 13: Orofacial Examination Chapter 14: Nasometry Chapter 15: Speech Aerodynamics of Cleft Palate Chapter 16: Videofluoroscopy and Other Forms of Radiography Chapter 17: Nasopharyngoscopy Part V:  Surgical Management of Clefts and Velopharyngeal Dysfunction Chapter 19: Orthognathic Surgery for Craniofacial Differences Chapter 20: Prosthetic Management Chapter 21: Speech Therapy: Making it Simple Glossary  Resources for Information and Support Appendix B: Selected Bibliography for Parents

title: The middle ear of cleft palate patients in their early teens: a literature study and preliminary file study.
abstract: Middle ear disease is a frequent problem in young children with cleft palate (CP) Less is known about otological status in the adolescent CP population. The aim of this study is to provide an overview of current knowledge in the literature concerning the aetiology of middle ear disease in CP patients and the middle ear status of older children with CP, and to compare the situation in our centre to this background through an assessment of the otological status of patients in our CP population.  A literature review was conducted to summarise current knowledge about middle ear status in CP patients. A retrospective analysis was performed of the medical records of 20 CP patients between the ages of 10 and 15 who were treated and followed at the University Hospitals Leuven. The available otological data, otoscopic findings, information about hearing and surgery performed was collected for each patient when aged three and above ten years.  Current knowledge indicates that middle ear function improves as CP patients get older. In our study, otoscopic appearance was acceptable in 63% of ears of children aged over ten years of age. Otitis media with effusion (OME) was far less frequent above this age than around the age of 3, with a decrease from 50% to 13% of all ears. Above the age of 10, tympanic perforations were present in 13% of ears and retraction of the tympanic membrane in 23%  Despite a very high incidence of OME in young CP patients, the long term otological prognosis is not necessarily sinister. A favourable natural evolution, diligent otological follow up, early diagnosis and treatment of middle ear disease with the use of tympanostomy tubes are the likely contributors to the acceptable otological result in older CP patients.

title: Sequelae of otitis media with effusion among children with cleft lip and/or cleft palate.
abstract: Otitis media with effusion (OME) is common among children with cleft palate, and may lead to such long term consequences as hearing loss, tympanic membrane retraction, and chronic otitis media (COM) In total, 104 children with cleft lip and/or palate treated for OME at our institution were reviewed. Mean duration of follow up was 6.9 years, and mean age at latest follow up was 9.6 years. The incidence of COM was 19% and the incidence of cholesteatoma was 1.9% Ears showing such long term sequelae of OME as hearing loss, tympanic membrane retraction, and chronic otitis media, were noted to have undergone a significantly greater number of ventilation tube insertions than ears not showing these sequalae. Our findings would suggest that a conservative approach to the management of OME in children with cleft palate is more likely to be beneficial in the long term.

title: Management of cleft lip and palate.
abstract: The complex nature of treatment for CL/P, a condition that requires a large multidisciplinary team treating patients from birth to maturity, has been outlined. Subjecting centres' outcomes to audit should precede heeding the current siren calls for paediatricians to refer children exclusively to a particular surgical speciality. A growing body of evidence has shown a close correlation between quality of outcome and the availability of high volume centralised care by dedicated teams, as has been proved and accepted for years in other fields such as surgery in infancy, childhood malignancies, and cystic fibrosis.

title: Audiologic and tympanometric findings in children with cleft lip and palate.
abstract: To evaluate the otologic and audiologic status of 50 children with repaired cleft lip, cleft palate, or both in Hacettepe University, Ankara, Turkey.  Audiometric and tympanometric evaluation of 100 ears in 50 children were performed. Hearing levels or 15 dB and middle ear pressures between 50 to +50 decaPascals were considered to be normal. Results were examined according to cleft type and laterality. The least and most affected frequencies were calculated. A simple evaluation of speech characteristics including nasal resonance, nasal air escape, and errors of articulation was also performed.  Sixty three of the 100 ears had normal hearing status, whereas 40 had normal middle ear pressures. No evidence was found to suggest that individual cleft type and laterality of the ear had any effect on hearing loss or middle ear disease. Two thirds of the patients had normal or acceptable degree of language skills.  The final hearing status of patients with cleft palate is a result of a combination of surgical correction, developmental factors, and treatment of middle ear disease. Early and aggressive ventilation tube placement is the standard of cleft care in many countries. Our long term hearing outcome is relatively good in a population not treated with routine insertion of ventilation tubes. The majority of patients also have satisfactory speech. Patients with cleft palate should have close follow up for middle ear disease, but further research is warranted to determine the aggressive usage of ventilation tubes.