Current notions of “hearing impairment,” as reflected in clinical audiological practice, do not acknowledge the needs of individuals who have normal hearing pure tone sensitivity but who experience auditory processing difficulties in everyday life that are indexed by reduced performance in other more sophisticated audiometric tests such as speech audiometry in noise or complex non-speech sound perception. This disorder, defined as “Auditory Processing Disorder” (APD) or “Central Auditory Processing Disorder” is classified in the current tenth version of the International Classification of diseases as H93.25 and in the forthcoming beta eleventh version. APDs may have detrimental effects on the affected individual, with low esteem, anxiety, and depression, and symptoms may remain into adulthood. These disorders may interfere with learning per se and with communication, social, emotional, and academic-work aspects of life. The objective of the present paper is to define a baseline European APD consensus formulated by experienced clinicians and researchers in this specific field of human auditory science. A secondary aim is to identify issues that future research needs to address in order to further clarify the nature of APD and thus assist in optimum diagnosis and evidence-based management. This European consensus presents the main symptoms, conditions, and specific medical history elements that should lead to auditory processing evaluation. Consensus on definition of the disorder, optimum diagnostic pathway, and appropriate management are highlighted alongside a perspective on future research focus.
Mutations in the connexin 26 gene (GJB2) are responsible for the major part of nonsyndromic autosomal recessive or apparently sporadic prelingual deafness in Caucasians (DFNB1). We screened 228 German hearing-impaired persons for mutations in the GJB2 gene by sequence analysis. Homozygous or compound heterozygous GJB2 mutations were detected in 38/228 (16.7%) of hearing impaired persons. The most frequently occurring mutation was the c.35delG mutation, which was found in 71.1% of the mutated alleles. The next frequent mutation detected in the group of hearing impaired persons was the c.101T>C mutation (9/76 alleles; 11.8%). One new mutation, c.567delA, was observed. We further studied the presence of a 10bp deletion in the 5' UTR of the GJB2 gene (c.-493del10) which was assumed to occur together with the c.101T>C mutation. Ten out of thirteen patients (76.9%) were found to be carriers of both the c.101T>C mutation and the 10bp variant and in 7/14 alleles a linkage disequilibrium between c.101T>C and the 10bp deletion was proven. In 4/14 alleles the linkage was ruled out and for the remaining 3 cases the phase determination was not possible. Seventy one controls were screened for the prevalence of Cx26 mutations and for the c.-493del10 variant. Heterozygosity frequency in the control group was for c.35delG 4.2%, for c.101T>C 1.4% and for c.-493del10 it was 5.6%.
After the differential diagnostic exclusion of peripheral hearing disorders and cognitive impairments, these three tests are sufficient to initially justify the clinical-diagnostic classification "(C)APD" in 8-11-year-old children taking into account only a relatively slight probability of error. Poor results in two of the three differentiating tests (discrepancy criterion of >1 SD of the reference population) are sufficient to support the diagnostic classification of an (C)APD.
The report was intended to provide up-to-date information about permanent mild hearing impairment (HI) in early childhood. Altogether 33/250 children with a permanent mild HI in the better ear (average at frequencies of 0.5, 1, 2, 4 kHz ≧25–39 dB HL) were found during the 7-year period from 1994 to 2001 in the Outpatient Clinic of the Department Phoniatrics/Pedaudiology, University of Göttingen. Twenty-two of 33 children had a mild HI in one or both ears. The loss was sensorineural in 73% of the children (88% bilateral, 12% unilateral), and conductive in 27% (67% bilaterally, 33% unilaterally) because of ear malformations or a suspicion of middle ear anomalies. Clinical data and test performances were presented for these four types of mild HI. Altogether, standardized language assessment demonstrated no delays in receptive and expressive vocabulary ability development on average, although the acquisition of two-word phrases in infancy was in mean delayed. Nonverbal intelligence was within the normal range, and on average the children did not perform less well in central auditory processing than normal children. Provided that no additional handicaps were present, mild HI did not adversely affect speech/language development if the children were fitted with hearing aids after early identification of HI.
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