Johnson et al.(3737 Marler JA, Sitcovsky JL, Mervis CB, Kistler DJ, Wightman FL. Auditory function and hearing loss in children and adults with Williams syndrome: Cochlear impairment in individuals with otherwise normal hearing. Am J Med Genet C Semin Med Genet. 2010;154C(2):249-65. http://dx.doi.org/10.1002/ajmg.c.30262. PMid:20425785. http://dx.doi.org/10.1002/ajmg.c.30262 ...
)
|
Cohort; Prospective; Cross-sectional |
9 individuals with WS |
Without information |
Pure-tone audiometry; TEOAE |
Cherniske et al.(3838 Zarchi O, Attias J, Raveh E, Basel-Vanagaite L, Saporta L, Gothelf D. A comparative study of hearing loss in two microdeletion syndromes: velocardiofacial (22q11.2 Deletion) and Williams (7q11.23 Deletion) Syndromes. J Pediatr. 2011;158(2):301-6. http://dx.doi.org/10.1016/j.jpeds.2010.07.056. PMid:20846670. http://dx.doi.org/10.1016/j.jpeds.2010....
)
|
Cohort; Prospective; Cross-sectional |
20 individuals with WS |
Mean of 38 years (30-51 years) |
Pure-tone audiometry |
Marler et al.(1919 Marler JA, Elfenbein JL, Ryals BM, Urban Z, Netzloff ML. Sensorineural hearing loss in children and adults with Williams syndrome. Am J Med Genet. 2005;138(4):318-27. http://dx.doi.org/10.1002/ajmg.a.30970. PMid:16222677. http://dx.doi.org/10.1002/ajmg.a.30970 ...
)
|
Cohort; Prospective; Cross-sectional |
27 individuals with WS |
Mean of 15 years (6-48 years) |
Pure-tone audiometry; DPOAE |
Gothelf et al.(2020 Gothelf D, Farber N, Raveh E, Apter A, Attias J. Hyperacusis in Williams syndrome: Characteristics and associated neuroaudiologic abnormalities. Neurology. 2006;66(3):390-5. http://dx.doi.org/10.1212/01.wnl.0000196643.35395.5f. PMid:16476938. http://dx.doi.org/10.1212/01.wnl.000019...
)
|
Cohort; Prospective; Cross-sectional |
49 individuals with WS; Individuals with DT (without information about sample size) |
WS: age group of 1-35 years; TD: paired by chronological age |
Ipsilateral acoustic reflexes; Pure-tone audiometry; DPOAE; BAEP |
Attias et al.(3939 Barozzi S, Soi D, Comiotto E, Borghi A, Gavioli C, Spreafico E, et al. Audiological findings in Williams syndrome: a study of 69 patients. Am J Med Genet. 2012;158A(4):759-71. http://dx.doi.org/10.1002/ajmg.a.35241. PMid:22411878. http://dx.doi.org/10.1002/ajmg.a.35241 ...
)
|
Cohort; Prospective; Cross-sectional |
21 individuals with WS; 21 individuals with DT |
WS: age group of 6-26 years; TD: paired by chronological age |
Ipsi- and contra-lateral acoustic reflexes; TEOAE with suppression |
Marler et al.(4040 Barozzi S, Soi D, Spreafico E, Borghi A, Comiotto E, Gagliardi C, et al. Audiological follow-up of 24 patients affected by Williams syndrome. Eur J Med Genet. 2013;56(9):490-6. http://dx.doi.org/10.1016/j.ejmg.2013.07.001. PMid:23886711. http://dx.doi.org/10.1016/j.ejmg.2013.0...
)
|
Cohort; Prospective; Cross-sectional |
81 individuals with WS; 14 individuals with DT |
WS 1 (43 individuals): mean of 12 years (5-17 years); WS 2 (38 individuals): mean of 31 years (18-59 years); TD: mean of 13 years (7-32 years) |
Tympanometry; Pure-tone audiometry; DPOAE |
Bedeschi et al.(4141 Attias J, Raveh E, Ben-Naftali NF, Zarchi O, Gothelf D. Hyperactive auditory efferent system and lack of acoustic reflexes in Williams syndrome. J Basic Clin Physiol Pharmacol. 2008;19(3-4):193-207. http://dx.doi.org/10.1515/JBCPP.2008.19.3-4.193. PMid:19025031. http://dx.doi.org/10.1515/JBCPP.2008.19...
)
|
Cohort; Retrospective; Cross-sectional |
13 individuals with WS |
Age group of 17-44 years |
Pure-tone audiometry; |
Paglialonga et al.(4242 Cherniske EM, Carpenter TO, Klaiman C, Young E, Bregman J, Insogna K, et al. Multisystem study of 20 older adults with Williams syndrome. Am J Med Genet. 2004;131(3):255-64. http://dx.doi.org/10.1002/ajmg.a.30400. PMid:15534874. http://dx.doi.org/10.1002/ajmg.a.30400 ...
)
|
Cohort; Prospective; Cross-sectional |
13 individuals with WS; 14 individuals with DT |
WS: age group of 7-23 years; DT: age group of 5-20 years |
TEOAE; (Tympanometry, acoustic reflex search, and pure-tone audiometry should be normal because they were used as inclusion criteria) |
Zarchi et al.(4343 Johnson LB, Comeau M, Clarke KD. Hyperacusis in Williams syndrome. J Otolaryngol. 2001;30(2):90-2. http://dx.doi.org/10.2310/7070.2001.20811. PMid:11770962. http://dx.doi.org/10.2310/7070.2001.208...
)
|
Cohort; Prospective; Cross-sectional |
44 individuals with WS; 23 individuals with DT |
WS: mean of 16 years (5-35 years); TD: mean of 17 years (6-38 years) |
Tympanometry; Ipsilateral acoustic reflexes; Pure-tone audiometry |
Barozzi et al.(4444 Bedeschi MF, Bianchi V, Colli AM, Natacci F, Cereda A, Milani D, et al. Clinical follow-up of young adults affected by Williams syndrome: experience of 45 Italian patients. Am J Med Genet. 2011;155(2):353-9. http://dx.doi.org/10.1002/ajmg.a.33819. PMid:21271653. http://dx.doi.org/10.1002/ajmg.a.33819 ...
)
|
Cohort; Prospective; Cross-sectional |
69 individuals with WS |
Mean of 11 years (2-30 years) |
Tympanometry; Contralateral acoustic reflexes; Pure-tone audiometry with visual or conventional reinforcement; TEOAE; BAEP |
Barozzi et al.(4545 Paglialonga A, Barozzi S, Brambilla D, Soi D, Cesarani A, Spreafico E, et al. Analysis of subtle auditory dysfunctions in young normal-hearing subjects affected by Williams syndrome. Int J Pediatr Otorhinolaryngol. 2014;78(11):1861-5. http://dx.doi.org/10.1016/j.ijporl.2014.08.010. PMid:25193583. http://dx.doi.org/10.1016/j.ijporl.2014...
)
|
Cohort; Prospective; Longitudinal (1st assessment; 2nd assessment after 5 years and 3rd assessment after 10 years) |
24 individuals with WS participated in the 1st and 2nd assessments, but only 10 of them participated in the 3rd assessment |
Mean of 8 years in the 1st assessment (5-14 years) |
Tympanometry; Contralateral acoustic reflexes; Pure-tone audiometry; TEOAE |
Paglialonga et al.(4646 Paglialonga A, Barozzi S, Brambilla D, Soi D, Cesarani A, Gagliardi C, et al. Cochlear active mechanisms in young normal-hearing subjects affected by Williams syndrome: Time-frequency analysis of otoacoustic emissions. Hear Res. 2011;272(1-2):157-67. http://dx.doi.org/10.1016/j.heares.2010.10.004. PMid:20969939. http://dx.doi.org/10.1016/j.heares.2010...
)
|
Cohort; Prospective; Cross-sectional |
21 ears of 13 individuals with WS; 13 individuals with DT |
WS: age group of 9-31 years; TD: paired by chronological age |
DPOAE; (Tympanometry, acoustic reflexes, and pure-tone audiometry should be normal because they were used as inclusion criteria) |