King-Kopetzky syndrome

King-Kopetzky syndrome is an auditory disability with appears clinically as a “normal” hearing threshold. It is also known as "obscure auditory dysfunction (OAD)"The role of a family history in King Kopetzky syndrome (Obscure Auditory Dysfunction). Stephens D., Zhao F.Acta Otolaryngol 2000; 120: 197-200.] or "auditory disability with normal hearing (ADN)", The main reported disability is difficulty in hearing speech in the presence of background noise. [http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=14570238&dopt=Abstract PubMed] ] this is in conjunction with the finding of normal hearing test results.

It was found that King Kopetzky syndrome patients will have a worse Social Hearing Handicap index (SHHI),indicating they suffer a significant degree of speech-hearing disability.Determinants of speech-hearing disability in King Kopetzky syndrome. Zhao F, Stephens D. Scand Audiol.1996;25(2):91-6.]

Causes

It seems that somatic anxiety and situations of stress may be determinants of speech-hearing disability.Auditory and psychological factors in ‘auditory disability with normal hearing’. King K, Stephens D. Scand Audiol. 1992;21(2):109-14.]

Some studies indicated an increased prevalence of a family history of hearing impairment in these patients. The pattern of results is suggestive that King-Kopetzky patients may be related to conditions of autosomal dominant inheritance.Audiometric configuration in non-syndromic genetic hearing loss. Liu XZ, Xu L, Newton V. J Audiol Med 3:99-106] Non-syndromic hearing impairment: unparalleled heterogeneity. Van Camp G, Willems PJ, Smith RJH (1997). Am J Genet 60:758-764.]

ubcategories

It was found that based on sensitized measures of auditory dysfunction and on psychological assessment, Subjects could be subdivided into seven subcategories [http://www.medscape.com/medline/abstract/10997453 Zhao F; Stephens D, Subcategories of patient with King-Kopetzky syndrome Br J Audiol .2000;34(4):241-56.] ] .

1- middle ear dysfunction

2- mild cochlear pathology

3- central/medial olivocochlear efferent system (MOCS) auditory dysfunction

4- purely psychological problems

5- multiple auditory pathologies

6- combined auditory dysfunction and psychological problems

7- unknown

Different subgroups may represent different pathogenic and aetiological factors. Thus, subcategorization provides further understanding of the basis of King-Kopetzky syndrome, and hence may guide the rehabilitative management of these patients.

Recent research

There has been some interest in testing that King-Kopetzky syndrome might be an early stage of late onset familial hearing impairment by Professor Dafydd Stephens, F Zhao and Angeles Espeso at the Welsh Hearing Institute, Cardiff University.

ee also

*Hearing impairment
*List of eponymous diseases
*Professor Dafydd Stephens

References