Abstract
After the definition of hearing loss, audiological grading scales defining the presence and severity of hearing loss are presented. An overview is provided of epidemiological estimates of childhood hearing loss and issues involved in their calculation. After specification of the stages of hearing development, the different types of behavioural responses to sound that develop in normal children are described. An overview of the normal stages of speech and language development in normal-hearing children is followed by the impact of different kinds and degrees of hearing loss on speech and language acquisition of hearing-impaired children.
Risk factors for permanent childhood hearing impairment are listed, and likely additional disabilities are referred to. Infections with neurotropic viruses presenting complications and immunisation strategies are highlighted. A further aetiological key aspect concerns ototoxic side effects of anticancer drugs, anti-infective agents, loop diuretics and ototopical medications, which are presented in context with audiological diagnostics, monitoring and post-therapeutic follow-up, therapeutic options and preventive strategies. A third core area is related to syndromic and non-syndromic forms of hereditary hearing loss. More than 60 hearing loss syndromes are specified with their affected genes and chromosomal regions. Clinical manifestations with possible impact on hearing, ear-specific anomalies, the type of hearing impairment and cognitive function are described.
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Orphanet (2009c) Jervell and Lange-Nielsen syndrome. Available via http://www.orpha.net/consor/cgi-bin/Disease_Search.php?lng=EN&data_id=12056&Disease_Disease_Search_diseaseGroup=Jervell-Lange-Nielsen-syndrome&Disease_Disease_Search_diseaseType=Pat&Disease(s)/group of diseases=Jervell-and-Lange-Nielsen-syndrome&title=Jervell-and-Lange-Nielsen-syndrome&search=Disease_Search_Simple. Accessed 4 Aug 2016
Orphanet (2009d) Usher syndrome. Available via http://www.orpha.net/consor/cgi-bin/Disease_Search.php?lng=EN&data_id=662&Disease_Disease_Search_diseaseGroup=Usher-syndrome&Disease_Disease_Search_diseaseType=Pat&Disease(s)/group of diseases=Usher-syndrome&title=Usher-syndrome&search=Disease_Search_Simple. Accessed 31 July 2016
Orphanet (2009e) FG syndrome phenotypic spectrum. Available via http://www.orpha.net/consor/cgi-bin/Disease_Search.php?lng=EN&data_id=1053&Disease_Disease_Search_diseaseGroup=FG-syndrome&Disease_Disease_Search_diseaseType=Pat&Disease(s)/group of diseases=FG-syndrome&title=FG-syndrome&search=Disease_Search_Simple. Accessed 2 Aug 2016
Orphanet (2009f) Maternally-inherited diabetes and deafness. Available via http://www.orpha.net/consor/cgi-bin/Disease_Search.php?lng=EN&data_id=7037&Disease_Disease_Search_diseaseGroup=Maternally-inherited-diabetes-and-deafness-&Disease_Disease_Search_diseaseType=Pat&Disease(s)/group of diseases=Maternally-inherited-diabetes-and-deafness&title=Maternally-inherited-diabetes-and-deafness&search=Disease_Search_Simple. Accessed 2 Aug 2016
Orphanet (2010) Mucopolysaccharidosis type 6. Available via http://www.orpha.net/consor/cgi-bin/Disease_Search.php?lng=EN&data_id=24&Disease_Disease_Search_diseaseGroup=Maroteaux-Lamy-syndrome&Disease_Disease_Search_diseaseType=Pat&Disease(s)/group%20of%20diseases=Mucopolysaccharidosis-type-6&title=Mucopolysaccharidosis-type-6&search=Disease_Search_Simple. Accessed 1 Aug 2016
Orphanet (2011a) Biotinidase deficiency. Available via http://www.orpha.net/consor/cgi-bin/Disease_Search.php?lng=EN&data_id=11267&Disease_Disease_Search_diseaseGroup=Biotinidase-deficiency&Disease_Disease_Search_diseaseType=Pat&Disease(s)/group of diseases=Biotinidase-deficiency&title=Biotinidase-deficiency&search=Disease_Search_Simple. Accessed 4 Aug 2016
Orphanet (2011b) Scheie syndrome. Available via http://www.orpha.net/consor/cgi-bin/Disease_Search.php?lng=EN&data_id=12382&Disease_Disease_Search_diseaseGroup=mucopolysaccharidosis&Disease_Disease_Search_diseaseType=Pat&Disease(s)/group%20of%20diseases=Scheie-syndrome&title=Scheie-syndrome&search=Disease_Search_Simple. Accessed 1 Aug 2016
Orphanet (2012a) 22q11.2 deletion syndrome. Available via http://www.orpha.net/consor/cgi-bin/Disease_Search.php?lng=EN&data_id=126&Disease_Disease_Search_diseaseGroup=Di-George-syndrome&Disease_Disease_Search_diseaseType=Pat&Disease(s)/group%20of%20diseases=22q11-2-deletion-syndrome&title=22q11-2-deletion-syndrome&search=Disease_Search_Simple. Accessed 27 July 2016
Orphanet (2012b) Wolf-Hirschhorn syndrome. Available via http://www.orpha.net/consor/cgi-bin/Disease_Search.php?lng=EN&data_id=147&Disease_Disease_Search_diseaseGroup=Wolf-Hirschhorn-syndrome&Disease_Disease_Search_diseaseType=Pat&Disease(s)/group%20of%20diseases=Wolf-Hirschhorn-syndrome&title=Wolf-Hirschhorn-syndrome&search=Disease_Search_Simple. Accessed 27 July 2016
Orphanet (2012c) Aniridia. Available via http://www.orpha.net/consor/cgi-bin/Disease_Search.php?lng=EN&data_id=6018&Disease_Disease_Search_diseaseGroup=aniridia&Disease_Disease_Search_diseaseType=Pat&Disease(s)/group%20of%20diseases=Aniridia&title=Aniridia&search=Disease_Search_Simple. Accessed 4 Mar 2017
Orphanet (2012d) Opitz G/BBB syndrome. Available via http://www.orpha.net/consor/cgi-bin/Disease_Search.php?lng=EN&data_id=3423&Disease_Disease_Search_diseaseGroup=BBB-G-syndrome&Disease_Disease_Search_diseaseType=Pat&Disease(s)/group%20of%20diseases=Opitz-G-BBB-syndrome&title=Opitz-G-BBB-syndrome&search=Disease_Search_Simple. Accessed 2 Aug 2016
Orphanet (2013a) Camurati-Engelmann disease. Available via http://www.orpha.net/consor/cgi-bin/Disease_Search.php?lng=EN&data_id=1551&Disease_Disease_Search_diseaseGroup=Camurati-Engelmann-disease&Disease_Disease_Search_diseaseType=Pat&Disease(s)/group of diseases=Camurati-Engelmann-disease&title=Camurati-Engelmann-disease&search=Disease_Search_Simple. Accessed 29 July 2016
Orphanet (2013b) Cleidocranial dysplasia. Available via http://www.orpha.net/consor/cgi-bin/Disease_Search.php?lng=EN&data_id=443&Disease_Disease_Search_diseaseGroup=Cleidocranial-dysplasia&Disease_Disease_Search_diseaseType=Pat&Disease(s)/group of diseases=Cleidocranial-dysplasia&title=Cleidocranial-dysplasia&search=Disease_Search_Simple. Accessed 31 July 2016
Orphanet (2013c) Crouzon disease. Available via http://www.orpha.net/consor/cgi-bin/Disease_Search.php?lng=EN&data_id=244&Disease_Disease_Search_diseaseGroup=Crouzon-disease&Disease_Disease_Search_diseaseType=Pat&Disease(s)/group of diseases=Crouzon-disease&title=Crouzon-disease&search=Disease_Search_Simple. Accessed 30 July 2016
Orphanet (2013d) Townes-Brocks syndrome. Available via http://www.orpha.net/consor/cgi-bin/Disease_Search.php?lng=EN&data_id=218&Disease_Disease_Search_diseaseGroup=Townes-Brocks-syndrome&Disease_Disease_Search_diseaseType=Pat&Disease(s)/group of diseases=Townes-Brocks-syndrome&title=Townes-Brocks-syndrome&search=Disease_Search_Simple. Accessed 29 July 2016
Orphanet (2013e) Mucopolysaccharidosis type 2. Available via http://www.orpha.net/consor/cgi-bin/Disease_Search.php?lng=EN&data_id=131&Disease_Disease_Search_diseaseGroup=Mucopolysaccharidosis-type-2&Disease_Disease_Search_diseaseType=Pat&Disease(s)/group of diseases=Mucopolysaccharidosis-type-2&title=Mucopolysaccharidosis-type-2&search=Disease_Search_Simple. Accessed 2 Aug 2016
Orphanet (2013f) Pendred syndrome. Available via http://www.orpha.net/consor/cgi-bin/Disease_Search.php?lng=EN&data_id=558&Disease_Disease_Search_diseaseGroup=Pendred-syndrome&Disease_Disease_Search_diseaseType=Pat&Disease(s)/group of diseases=Pendred-syndrome&title=Pendred-syndrome&search=Disease_Search_Simple. Accessed 4 Aug 2016
Orphanet (2013g) Mohr-Tranebjaerg syndrome. Available via http://www.orpha.net/consor/cgi-bin/Disease_Search.php?lng=EN&data_id=10691&Disease_Disease_Search_diseaseGroup=Mohr-Tranebjaerg-syndrome&Disease_Disease_Search_diseaseType=Pat&Disease(s)/group of diseases=Mohr-Tranebjaerg-syndrome&title=Mohr-Tranebjaerg-syndrome&search=Disease_Search_Simple. Accessed 4 Aug 2016
Orphanet (2013h) X-linked agammaglobulinemia. Available via http://www.orpha.net/consor/cgi-bin/Disease_Search.php?lng=EN&data_id=142&Disease_Disease_Search_diseaseGroup=X-linked-agammaglobulinemia&Disease_Disease_Search_diseaseType=Pat&Disease(s)/group%20of%20diseases=X-linked-agammaglobulinemia&title=X-linked-agammaglobulinemia&search=Disease_Search_Simple. Accessed 4 Mar 2017
Orphanet (2014a) Down syndrome. Available via http://www.orpha.net/consor/cgi-bin/Disease_Search.php?lng=EN&data_id=116&Disease_Disease_Search_diseaseGroup=M%2D%2DDown&Disease_Disease_Search_diseaseType=Pat&Disease(s)/group%20of%20diseases=Down-syndrome&title=Down-syndrome&search=Disease_Search_Simple. Accessed 27 July 2016
Orphanet (2014b) Goldenhar syndrome. Available via http://www.orpha.net/consor/cgi-bin/Disease_Search.php?lng=EN&data_id=499&Disease_Disease_Search_diseaseGroup=Goldenhar-syndrome&Disease_Disease_Search_diseaseType=Pat&Disease(s)/group of diseases=Goldenhar-syndrome&title=Goldenhar-syndrome&search=Disease_Search_Simple. Accessed 28 July 2016
Orphanet (2014c) Crouzon syndrome-acanthosis nigricans syndrome. Available via http://www.orpha.net/consor/cgi-bin/Disease_Search.php?lng=EN&data_id=12205&Disease_Disease_Search_diseaseGroup=Crouzon-syndrome-acanthosis-nigricans-syndrome&Disease_Disease_Search_diseaseType=Pat&Disease(s)/group of diseases=Crouzon-syndrome-acanthosis-nigricans-syndrome&title=Crouzon-syndrome-acanthosis-nigricans-syndrome&search=Disease_Search_Simple. Accessed 30 July 2016
Orphanet (2014d) Facioscapulohumeral dystrophy. Available via http://www.orpha.net/consor/cgi-bin/Disease_Search.php?lng=EN&data_id=62&Disease_Disease_Search_diseaseGroup=Facioscapulohumeral-muscular-dystrophy&Disease_Disease_Search_diseaseType=Pat&Disease(s)/group%20of%20diseases=Facioscapulohumeral-dystrophy&title=Facioscapulohumeral-dystrophy&search=Disease_Search_Simple. Accessed 1 May 2017
Orphanet (2014e) Muckle-Wells syndrome. Available via http://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=GB&Expert=575. Accessed 1 May 2017
Orphanet (2014f) Schinzel-Giedion syndrome. Available via http://www.orpha.net/consor/cgi-bin/Disease_Search.php?lng=EN&data_id=2807&Disease_Disease_Search_diseaseGroup=Schinzel-Giedion-syndrome&Disease_Disease_Search_diseaseType=Pat&Disease(s)/group of diseases=Schinzel-Giedion-syndrome&title=Schinzel-Giedion-syndrome&search=Disease_Search_Simple. Accessed 27 July 2016
Orphanet (2014g) Treacher-Collins syndrome. Available via http://www.orpha.net/consor/cgi-bin/Disease_Search.php?lng=EN&data_id=293&Disease_Disease_Search_diseaseGroup=Treacher-Collins-&Disease_Disease_Search_diseaseType=Pat&Disease(s)/group of diseases=Treacher-Collins-syndrome&title=Treacher-Collins-syndrome&search=Disease_Search_Simple. Accessed 29 July 2016
Orphanet (2014h) Alström syndrome. Available via http://www.orpha.net/consor/cgi-bin/Disease_Search.php?lng=EN&data_id=1328&Disease_Disease_Search_diseaseGroup=Alstrom-syndrome&Disease_Disease_Search_diseaseType=Pat&Disease(s)/group of diseases=Alstrom-syndrome&title=Alstrom-syndrome&search=Disease_Search_Simple. Accessed 1 Aug 2016
Orphanet (2014i) Xeroderma pigmentosum-Cockayne syndrome complex. Available via http://www.orpha.net/consor/cgi-bin/Disease_Search.php?lng=EN&data_id=18903&Disease_Disease_Search_diseaseType=ORPHA&Disease_Disease_Search_diseaseGroup=220295&Disease(s)/group%20of%20diseases=XP-CS-complex&title=XP-CS-complex&search=Disease_Search_Simple. Accessed 1 May 2017
Orphanet (2014j) Primary ciliary dyskinesia. Available via http://www.orpha.net/consor/cgi-bin/Disease_Search.php?lng=EN&data_id=665&Disease_Disease_Search_diseaseGroup=Primary-Ciliary-Dyskinesia&Disease_Disease_Search_diseaseType=Pat&Disease(s)/group%20of%20diseases=Primary-ciliary-dyskinesia&title=Primary-ciliary-dyskinesia&search=Disease_Search_Simple. Accessed 1 Aug 2016
Orphanet (2014k) Hurler syndrome. Available via http://www.orpha.net/consor/cgi-bin/Disease_Search.php?lng=EN&data_id=12381&Disease_Disease_Search_diseaseGroup=Mucopolysaccharidosis&Disease_Disease_Search_diseaseType=Pat&Disease(s)/group of diseases=Hurler-syndrome&title=Hurler-syndrome&search=Disease_Search_Simple. Accessed 31 July 2016
Orphanet (2014l) Kearns-Sayre syndrome. Available via http://www.orpha.net/consor/cgi-bin/Disease_Search.php?lng=EN&data_id=61&Disease_Disease_Search_diseaseGroup=Kearns-Sayre-Syndrome-&Disease_Disease_Search_diseaseType=Pat&Disease(s)/group of diseases=Kearns-Sayre-syndrome&title=Kearns-Sayre-syndrome&search=Disease_Search_Simple. Accessed 2 Aug 2016
Orphanet (2015a) CHARGE syndrome. Available via http://www.orpha.net/consor/cgi-bin/Disease_Search.php?lng=EN&data_id=110&Disease_Disease_Search_diseaseGroup=CHARGE-syndrome&Disease_Disease_Search_diseaseType=Pat&Disease(s)/group of diseases=CHARGE-syndrome&title=CHARGE-syndrome&search=Disease_Search_Simple. Accessed 28 July 2016
Orphanet (2015b) Marshall syndrome. Available via http://www.orpha.net/consor/cgi-bin/Disease_Search.php?lng=EN&data_id=540&Disease_Disease_Sea rch_diseaseGroup=Marshall-syndrome&Disease_Disease_Search_diseaseType=Pat&Disease(s)/group of diseases=Marshall-syndrome&title=Marshall-syndrome&search=Disease_Search_Simple. Accessed 28 July 2016
Orphanet (2015c) Waardenburg syndrome. Available via http://www.orpha.net/consor/cgi-bin/Disease_Search.php?lng=EN&data_id=663&Disease_Disease_Search_diseaseGroup=Waardenburg-syndrome&Disease_Disease_Search_diseaseType=Pat&Disease(s)/group of diseases=Waardenburg-syndrome&title=Waardenburg-syndrome&search=Disease_Search_Simple. Accessed 29 July 2016
Orphanet (2015d) Maternal phenylketonuria. Available via http://www.orpha.net/consor/www/cgi-bin/OC_Exp.php?lng=EN&Expert=2209. Accessed 3 June 2017
Orphanet (2015e) Refsum disease. Available via http://www.orpha.net/consor/cgi-bin/Disease_Search.php?lng=EN&data_id=381&Disease_Disease_Search_diseaseGroup=Refsum-disease&Disease_Disease_Search_diseaseType=Pat&Disease(s)/group of diseases=Refsum-disease&title=Refsum-disease&search=Disease_Search_Simple. Accessed 4 Aug 2016
Orphanet (2015f) Otopalatodigital syndrome type 1. Available via http://www.orpha.net/consor/cgi-bin/Disease_Search.php?lng=EN&data_id=12059&Disease_Disease_Search_diseaseGroup=Otopalatodigital-syndrome-type-1&Disease_Disease_Search_diseaseType=Pat&Disease(s)/group of diseases=Otopalatodigital-syndrome-type-1&title=Otopalatodigital-syndrome-type-1&search=Disease_Search_Simple. Accessed 2 Aug 2016
Orphanet (2015g) MELAS. Available via http://www.orpha.net/consor/cgi-bin/Disease_Search.php?lng=EN&data_id=63&Disease_Disease_Search_diseaseGroup=MELAS&Disease_Disease_Search_diseaseType=Pat&Disease(s)/group of diseases=MELAS&title=MELAS&search=Disease_Search_Simple. Accessed 2 Aug 2016
Orphanet (2015h) Fetal hydantoin syndrome. Available via http://www.orpha.net/consor/cgi-bin/Disease_Search.php?lng=EN&data_id=1875&Disease_Disease_Search_diseaseGroup=Fetal-Hydantoin-Syndrome&Disease_Disease_Search_diseaseType=Pat&Disease(s)/group%20of%20diseases=Fetal-hydantoin-syndrome&title=Fetal-hydantoin-syndrome&search=Disease_Search_Simple. Accessed 4 Sept 2016
Orphanet (2016) Langer-Giedion syndrome. Available via http://www.orpha.net/consor/cgi-bin/Disease_Search.php?lng=EN&data_id=526&Disease_Disease_Search_diseaseGroup=Langer-Giedion-syndrome&Disease_Disease_Search_diseaseType=Pat&Disease(s)/group of diseases=Langer-Giedion-syndrome&title=Langer-Giedion-syndrome&search=Disease_Search_Simple. Accessed 27 July 2016
Orphanet (2017) Muenke syndrome. Available via http://www.orpha.net/consor/cgi-bin/Disease_Search.php?lng=EN&data_id=10716&Disease_Disease_Search_diseaseGroup=Muenke-syndrome&Disease_Disease_Search_diseaseType=Pat&Disease(s)/group of diseases=Muenke-syndrome&title=Muenke-syndrome&search=Disease_Search_Simple. Accessed 30 July 2016
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am Zehnhoff-Dinnesen, A. et al. (2020). Basics of Disorders of Hearing Development. In: am Zehnhoff-Dinnesen, A., Wiskirska-Woznica, B., Neumann, K., Nawka, T. (eds) Phoniatrics I. European Manual of Medicine. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-46780-0_14
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