Causes of permanent childhood hearing impairment
β Scribed by Anna M.H. Korver; Ronald J.C. Admiraal; Sarina G. Kant; Friedo W. Dekker; Capi C. Wever; Henricus P.M. Kunst; Johan H.M. Frijns; Anne Marie Oudesluys -Murphy; on behalf of the DECIBEL-collaborative study group
- Publisher
- John Wiley and Sons
- Year
- 2011
- Tongue
- English
- Weight
- 242 KB
- Volume
- 121
- Category
- Article
- ISSN
- 0023-852X
No coin nor oath required. For personal study only.
β¦ Synopsis
Abstract
Introduction:
The causes of Permanent Childhood Hearing Impairment (PCHI) are often quoted as being hereditary in 50%, acquired in 25%, and unknown in 25% of cases. Interest in the causes of PCHI has grown recently due to increasing diagnostic possibilities. We investigated the evidence for the reported distribution of these causes.
Methods:
Populationβbased study and a systematic review. Inclusion criteria for populationβbased study: children born between 2003 and 2005, resident in The Netherlands at birth, known at an Audiology Center with PCHI at the age of 3β5 years. The causes of PCHI were determined prospectively by detection of congenital cytomegalovirus on dried blood spots and/or genetic diagnostic investigations in addition to reviewing data from medical records. A systematic review was carried out using three terms (hearing loss, infant, and etiology) and limited to articles published between January 1997 and July 2009. Main outcome measures were: the (weighted) proportions of the various causes of PCHI following diagnostic investigations.
Results:
In the studyβpopulation (n = 185) a hereditary cause was found in 38.9%, acquired cause in 29.7%, miscellaneous cause in 7.1%, and the cause remained unknown in 24.3%. The systematic review of the literature (n = 9 articles) resulted in a weighted mean of 30.4% hereditary, 19.2% acquired, and 48.3% unknown causes of PCHI.
Discussion:
The systematic review and the results of the populationβbased study provided little support for the generally accepted distribution of causes of PCHI. Laryngoscope, 2011
π SIMILAR VOLUMES
## Abstract Nongenetic as well as genetic etiologies must be explored in the child with identified hearing loss. Graduates of the neonatal intensive care unit are at increased risk for developing hearing loss due to hypoxia, hyperbilirubinemia, very low birth weight, and ototoxic medications. Altho
We have identified a consanguineous family from Morocco segregating autosomal recessive congenital progressive hearing loss (ARNSHL) and retinal degeneration. Detailed clinical investigation of the six siblings revealed combined severe cone-rod dystrophy (CORD) and severe/profound hearing impairment
Mutations of the connexin 26 gene (GJB2) were studied in 365 apparently unrelated individuals with profound nonsyndromic, sensorineural hearing impairment from Ghana, West Africa. Among 121 mutated chromosomes found, 110 carried the previously described R143W mutation. A total of 6 novel mutations:
The populations formed by the pupils of the eleven Dutch institutions for visually handicapped children on the first of January 1980 were investigated, repeating the investigation of the International Association for Prevention of Blindness in 1970. The total number of pupils was nearly the same in