ABSTRACT
The Deutsches Zentralregister für kindliche Hörstörungen (DZH = German Registry for Hearing Loss in Children) has so far (30.6.2000) entered data records of 4,027 permanently hearing impaired children. These records include data on a total of 151 pairs of siblings. In seven cases a third sibling was registered. An analysis of the data of siblings with different dates of birth shows that the diagnosis for older siblings was delayed 2 years on average compared to the younger siblings (mean 23.6 months, 95% confidence interval 19.3-27.8 months). The degree of hearing loss, which crucially determines the average age at which a child is diagnosed, correlates statistically significantly with pairs of siblings, so that misleading results can be almost totally avoided. In 11 of 17 cases of twins and triplets, the diagnosis was pronounced simultaneously for both siblings; in the remaining 6 cases, one sibling was diagnosed later. It has become apparent that younger siblings of hearing-impaired children profit from the concomitant routine diagnostic investigation of siblings of hearing-impaired children nowadays common in many institutions, but avoidable delays nevertheless still occur, as shown by the data for twins and triplets. Since in approximately 18% of the registered children with one or more siblings one of the brothers or sisters is likewise hearing-impaired, consistent family investigation is recommended.
Subject(s)
Deafness/genetics , Hearing Loss, Sensorineural/genetics , Age Factors , Child, Preschool , Deafness/diagnosis , Diseases in Twins/genetics , Female , Hearing Loss, Sensorineural/diagnosis , Humans , Infant , Infant, Newborn , Male , Triplets/geneticsABSTRACT
Since April 1996 the German Registry for Hearing Loss in Children has been collecting and recording various data on permanent hearing loss in children. Up to now, the data of 3882 children have been gathered nation-wide. A particularly remarkable aspect of data so far collected is the alarmingly high age at the time of diagnosis (mild hearing loss is on average only diagnosed at 6; 2 years, moderate loss at 4; 4 years, severe at 2; 5 years and profound with 1; 9 years). The German Registry for Hearing Loss in Children provides the first reliable source of epidemiological data on permanent hearing loss in children in Germany. A constantly growing number of currently 112 co-operating partners takes part in the data acquisition. The periodical reports of results to the co-operating partners and the first publications have heightened the awareness of the importance of early diagnosis and therapy. A minimum standard for patient history was established by the primary examination report. Thus, an important contribution has been made to the prevention of avoidable secondary damage.
Subject(s)
Hearing Disorders/epidemiology , Registries , Child , Child, Preschool , Female , Germany/epidemiology , Hearing Disorders/diagnosis , Hearing Disorders/etiology , Humans , Incidence , Male , Severity of Illness Index , Surveys and QuestionnairesABSTRACT
OBJECTIVE: This prospective study reports on the prevalence of hearing impairment in an at-risk neonatal intensive care unit (NICU) population. DESIGN: From 1990 to 1998, 1062 neonates were screened with the use of transitory evoked otoacoustic emissions (TEOAE) and brainstem evoked response audiometry (BERA). RESULTS: 934 infants passed the primary screen for both ears, 75 for one ear, adding up to 95%. 17 infants (1.6%) were lost to follow-up. In fourteen infants (1.3%), bilateral hearing impairment above 30 dB was confirmed. While all children with hearing impairment belonged to the group of 862 children receiving aminoglycosides, only one of them presented no other risk factors. In twelve of the hearing impaired children other anamnestic factors, i.e. dysmorphism, prenatal rubella or cytomegaly, family history of hearing loss or severe peri- and postnatal complications seem to be more probable causes of the identified hearing loss. In one of these children, delayed onset or progression of hearing loss is suspected. CONCLUSIONS: From our data, aminoglycosides are not an important risk factor for hearing impairment, when serum levels are continuously monitored, as in our cohort. After adjustment for other risk factors, birth weight between 1000 gr and 1500 gr and a gestational age between 29 and 31 weeks were no predictive markers for hearing impairment. It might be speculated that the improved medical treatment in a NICU reduces the probability of hearing impairment for those two groups. Conductive hearing loss as a possible additional cause for hearing impairment was not studied in detail, but the high percentage of malformations detected (four out of fourteen hearing impaired infants) demands further monitoring, close follow-up, adequate treatment and counselling.
Subject(s)
Audiometry, Pure-Tone , Deafness/congenital , Infant, Premature, Diseases/diagnosis , Neonatal Screening , Otoacoustic Emissions, Spontaneous , Auditory Threshold/physiology , Brain Stem/physiopathology , Deafness/diagnosis , Deafness/physiopathology , Female , Humans , Infant, Newborn , Infant, Premature, Diseases/physiopathology , Intensive Care Units, Neonatal , Male , Predictive Value of Tests , Prospective Studies , Risk FactorsABSTRACT
The results of international investigations on connatally acquired hearing loss are compared with the data of the German Registry on Childhood Hearing Loss (4058 cases). The connatal hearing disorders have shown a notable change in the last years regarding to aetiology and prevalence. In contrast to countries of the third world in developed nations the prevalence of permanent childhood hearing loss has been reduced down to 1 in 1.000 births. The results let assume a prevalence of approximately 1:1.200 births in Germany. For instance the number of rubella embryopathia decreased effectively. In contrast CMV infections and alcohol fetopathia are playing an increasing role. In the patients of the German Registry on Childhood Hearing Loss the percentage of certainly progressive hearing loss is 10.3 within the 4058 children with permanent hearing impairment. Diagnostic procedures first of all for the early diagnosis of CMV but also of toxoplasmosis are considerable because these infections may result in treatable hearing loss. Also consequent hearing tests are demanded in children with alcohol fetopathia.
Subject(s)
Deafness/congenital , Pregnancy Complications, Infectious/diagnosis , Prenatal Exposure Delayed Effects , Child , Child, Preschool , Cross-Sectional Studies , Deafness/epidemiology , Developing Countries , Female , Humans , Infant , Infant, Newborn , Male , Pregnancy , Pregnancy Complications, Infectious/epidemiology , Risk FactorsABSTRACT
Since 1994, the German Registry for hearing Loss in Children has registered data of 1500 children and by now can present results concerning the age at diagnosis of permanent hearing loss in children in Germany. The mean age at diagnosis is still very high. There is a strong correlation between age at diagnosis and degree of hearing loss, i.e., severe and profound hearing loss, is diagnosed distinctly earlier than mild and moderate hearing loss. On average, mild hearing loss is diagnosed with 6.2 years, moderate h.l. with 4.4 years, severe hearing loss with 2.5 years and profound hearing loss with 1.9 years. This corresponds with the results of regional German studies. At least regionally, in other European countries the age at diagnosis is known to be distinctly lower. In 36% of the children registered in Germany the delay between first suspicion and diagnosis of permanent hearing loss is 1 year or more.
Subject(s)
Deafness/diagnosis , Hearing Disorders/diagnosis , Age Factors , Child , Child, Preschool , Deafness/etiology , Deafness/prevention & control , Female , Germany , Hearing Disorders/etiology , Hearing Disorders/prevention & control , Humans , Infant , Male , Mass Screening , RegistriesABSTRACT
The German Registry for Hearing Loss in Children (DZH) processes nationwide data from audiological centers. Coping with the accrued data and its subsequent management and analysis requires a high degree of security and control. To establish a nationwide registry it is necessary at an early stage to take into consideration the legal requirements of the participating states. Use of the DZH as an example demonstrates how a pragmatic solution can be reached. Special issues concerning data collection, transfer, storage and deletion, coding strategies to ensure anonymity, checking for duplicate entries, data separation, and automated data analysis and data protection are explained.
Subject(s)
Computer Security/legislation & jurisprudence , Hearing Disorders/epidemiology , Medical Records Systems, Computerized/legislation & jurisprudence , Registries/statistics & numerical data , Adolescent , Adult , Child , Child, Preschool , Data Collection/legislation & jurisprudence , Female , Germany/epidemiology , Hearing Disorders/etiology , Humans , Infant , Infant, Newborn , Male , Patient Care TeamABSTRACT
OBJECTIVE: This prospective study reports on the prevalence of hearing impairment in an at-risk neonatal intensive care unit (NICU) population. DESIGN: From 1990 to 1997, 942 neonates were screened with transient evoked otoacoustic emissions (TEOAE) and brainstem evoked response audiometry (BERA). RESULTS: 835 Infants passed the primary screen for both ears, 57 for one ear, adding up to 94.7%. Seventeen infants (1.9%) were lost to follow-up. In thirteen infants (1.4%), bilateral hearing impairment above 30 dB was confirmed. While all children with hearing impairment belonged to the group of 820 children receiving aminoglycosides, only one presented no other risk factors. In 11 of the hearing impaired children other anamnestic factors, i.e. dysmorphism, prenatal rubella or cytomegaly, family history of hearing loss or severe peri- and postnatal complications seem to be more probable causes of the identified hearing loss. CONCLUSIONS: From our data, aminoglycosides seem not to be an important risk factor for communication related hearing impairment, when serum levels are continuously monitored, as occurred in our cohort. After adjustment for other risk factors, birth weight between 1000 and 1500 g and a gestational age between 29 and 31 weeks were no predictive markers for hearing impairment. It might be speculated that the improved medical treatment in a Neonatal Intensive Care Unit (NICU) reduces the probability of hearing impairment for those two groups. Conductive hearing loss as a possible additional cause for hearing impairment was not studied in detail, but the high percentage of malformations detected (four out of 13 hearing impaired infants) demands further monitoring, close follow-up, counselling and adequate treatment.
