Apert syndrome is one of the craniosynostosis syndromes and is commonly associ ated with conductive hearing loss, but there are contradicting reports regarding the cause. Retrospective analysis of case notes of Apert ...Apert syndrome is one of the craniosynostosis syndromes and is commonly associ ated with conductive hearing loss, but there are contradicting reports regarding the cause. Retrospective analysis of case notes of Apert syndrome patients seen between 1970 and 2003 was therefore undertaken. Seventy case notes were obtaine d; 59%were males. The incidence of congenital hearing impairment was 3.0%. Alm ost all had otitismedia with effusion, which tends to persist into adulthood. Mo re than 56%developed permanent low frequency conductive hearing loss by 10-20 years of age.展开更多
Isolated case reports in which symptomatic hearing loss develops suddenly during the course of inflammatory bowel disease (IBD) have been reported, but the presence of subclinical sensorineural hearing loss (SNHL) ass...Isolated case reports in which symptomatic hearing loss develops suddenly during the course of inflammatory bowel disease (IBD) have been reported, but the presence of subclinical sensorineural hearing loss (SNHL) associated with IBD has been investigated in only two preliminary studies.In order to research this further, we aimed to investigate the presence of subclinical SNHL in IBD by comparison with a control group and to examine possible relations between the bowel disease parameters and hearing loss.Otoscopy, tympanometry, and pure tone audiometry were carried out in 39 patients with IBD (21 Crohn’s disease [CD], 18 ulcerative colitis [UC]) and 25 healthy ageand sex-matched controls. All patients and control subjects had normal otoscopy findings and tympanometry was unremarkable, excluding middle ear disease and conductive hearing loss. Analysis of each frequency examined showed that the average hearing thresholds were increased significantly in the study group compared to those of the control group at higher frequencies (2, 4, and 8 kHz). When these parameters were compared with the control group according to subgroups of IBD, a significant difference was determined for the UC group at frequencies of 2, 4, and 8 kHz and for the CD group only at the frequency of 4 kHz. Although there was a trend of increment in SNHL as the age of the patient and duration and extent of UC increased, no significant correlation was observed between SNHL and these parameters or sex, activity, involvement site, medication history of IBD, and coexistence of other extraintestinal manifestations. In conclusion, it was demonstrated that a subclinical SNHL may be associated with UC and somewhat with CD, affecting mainly the high frequencies. In light of this finding, it may be advisable to investigate labyrinth functions as well as other extraintestinal manifestations in patients with IBD.展开更多
Objectives:To (a) study the prevalence of hearing impairment in a cohort of very low birthweight (VLBW) infants and (b) evaluate the effectiveness of transient evoked otoacoustic emissions(TEOAE) as a first stage in-h...Objectives:To (a) study the prevalence of hearing impairment in a cohort of very low birthweight (VLBW) infants and (b) evaluate the effectiveness of transient evoked otoacoustic emissions(TEOAE) as a first stage in-hospital hearing screening tool in this population. Study design:The study group was a cohort of 346 VLBW infants born in 1998—2000 at The Sheba Medical Center. The prevalence of hearing impairment in the study group was compared with that of all other newborn infants participating in a universal newborn hearing screening programme during the same period. To evaluate the effectiveness of TEOAE,a control group of 1205 healthy newborns who had no known risk factors for hearing impairment was selected. The results and follow up of hearing screening for these infants were examined retrospectively. Results:Only one VLBW infant (0.3%) was diagnosed with bilateral sensory-neural hearing loss. In addition,nine infants (2.7%) were diagnosed with conductive hearing loss. Bronchopulmonary dysplasia and low Apgar score were the most significant factors for predicting the occurrence of conductive hearing loss. The percentage of VLBW infants who successfully passed the in hospital TEOAE screening was 87.2,compared with 92.2%in the full term control group. No false negative cases were detected on follow up. Conclusions:The study shows a low incidence of sensory-neural hearing loss in a cohort of VLBW infants and a relatively high incidence of conductive hearing loss. TEOAE screening was found to be an effective first stage in-hospital hearing screening tool in this population.展开更多
文摘Apert syndrome is one of the craniosynostosis syndromes and is commonly associ ated with conductive hearing loss, but there are contradicting reports regarding the cause. Retrospective analysis of case notes of Apert syndrome patients seen between 1970 and 2003 was therefore undertaken. Seventy case notes were obtaine d; 59%were males. The incidence of congenital hearing impairment was 3.0%. Alm ost all had otitismedia with effusion, which tends to persist into adulthood. Mo re than 56%developed permanent low frequency conductive hearing loss by 10-20 years of age.
文摘Isolated case reports in which symptomatic hearing loss develops suddenly during the course of inflammatory bowel disease (IBD) have been reported, but the presence of subclinical sensorineural hearing loss (SNHL) associated with IBD has been investigated in only two preliminary studies.In order to research this further, we aimed to investigate the presence of subclinical SNHL in IBD by comparison with a control group and to examine possible relations between the bowel disease parameters and hearing loss.Otoscopy, tympanometry, and pure tone audiometry were carried out in 39 patients with IBD (21 Crohn’s disease [CD], 18 ulcerative colitis [UC]) and 25 healthy ageand sex-matched controls. All patients and control subjects had normal otoscopy findings and tympanometry was unremarkable, excluding middle ear disease and conductive hearing loss. Analysis of each frequency examined showed that the average hearing thresholds were increased significantly in the study group compared to those of the control group at higher frequencies (2, 4, and 8 kHz). When these parameters were compared with the control group according to subgroups of IBD, a significant difference was determined for the UC group at frequencies of 2, 4, and 8 kHz and for the CD group only at the frequency of 4 kHz. Although there was a trend of increment in SNHL as the age of the patient and duration and extent of UC increased, no significant correlation was observed between SNHL and these parameters or sex, activity, involvement site, medication history of IBD, and coexistence of other extraintestinal manifestations. In conclusion, it was demonstrated that a subclinical SNHL may be associated with UC and somewhat with CD, affecting mainly the high frequencies. In light of this finding, it may be advisable to investigate labyrinth functions as well as other extraintestinal manifestations in patients with IBD.
文摘Objectives:To (a) study the prevalence of hearing impairment in a cohort of very low birthweight (VLBW) infants and (b) evaluate the effectiveness of transient evoked otoacoustic emissions(TEOAE) as a first stage in-hospital hearing screening tool in this population. Study design:The study group was a cohort of 346 VLBW infants born in 1998—2000 at The Sheba Medical Center. The prevalence of hearing impairment in the study group was compared with that of all other newborn infants participating in a universal newborn hearing screening programme during the same period. To evaluate the effectiveness of TEOAE,a control group of 1205 healthy newborns who had no known risk factors for hearing impairment was selected. The results and follow up of hearing screening for these infants were examined retrospectively. Results:Only one VLBW infant (0.3%) was diagnosed with bilateral sensory-neural hearing loss. In addition,nine infants (2.7%) were diagnosed with conductive hearing loss. Bronchopulmonary dysplasia and low Apgar score were the most significant factors for predicting the occurrence of conductive hearing loss. The percentage of VLBW infants who successfully passed the in hospital TEOAE screening was 87.2,compared with 92.2%in the full term control group. No false negative cases were detected on follow up. Conclusions:The study shows a low incidence of sensory-neural hearing loss in a cohort of VLBW infants and a relatively high incidence of conductive hearing loss. TEOAE screening was found to be an effective first stage in-hospital hearing screening tool in this population.
