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Delayed brainstem auditory evoked response conduction time

MedGen UID:
870862
Concept ID:
C4025322
Finding
Synonym: Prolonged brainstem auditory evoked potentials
 
HPO: HP:0004466

Definition

An abnormal increase (delay) in the conduction time of the brainstem auditory-evoked response. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVDelayed brainstem auditory evoked response conduction time

Conditions with this feature

Hypomyelination and Congenital Cataract
MedGen UID:
501134
Concept ID:
C1864663
Congenital Abnormality
Hypomyelination and congenital cataract (HCC) is usually characterized by bilateral congenital cataracts and normal psychomotor or only mildly delayed development in the first year of life, followed by slowly progressive neurologic impairment manifest as ataxia, spasticity (brisk tendon reflexes and bilateral extensor plantar responses), and mild-to-moderate cognitive impairment. Dysarthria and truncal hypotonia are observed. Cerebellar signs (truncal titubation and intention tremor) and peripheral neuropathy (muscle weakness and wasting of the legs) are present in the majority of affected individuals. Seizures can occur. Cataracts may be absent in some individuals.
Charcot-Marie-Tooth disease type 4C
MedGen UID:
356581
Concept ID:
C1866636
Disease or Syndrome
SH3TC2-related hereditary motor and sensory neuropathy (SH3TC2-HMSN) is a demyelinating neuropathy characterized by severe spine deformities (scoliosis or kyphoscoliosis) and foot deformities (pes cavus, pes planus, or pes valgus) that typically present in the first decade of life or early adolescence. Other findings can include cranial nerve involvement (most commonly tongue involvement, facial weakness/paralysis, hearing impairment, dysarthria) and respiratory problems.
Hypomyelinating leukodystrophy 13
MedGen UID:
896545
Concept ID:
C4225170
Disease or Syndrome
Hypomyelinating leukodystrophy-13 is an autosomal recessive neurodegenerative disorder characterized by infantile onset of delayed psychomotor development, axial hypotonia, and spasticity associated with delayed myelination and periventricular white matter abnormalities on brain imaging. More variable neurologic deficits, such as visual impairment, may also occur. Some patients may experience cardiac failure during acute illness (summary by Edvardson et al., 2016). For a general phenotypic description and a discussion of genetic heterogeneity of HLD, see 312080.

Professional guidelines

PubMed

Findlen UM, Hounam GM, Alexy E, Adunka OF
Ear Hear 2019 May/Jun;40(3):651-658. doi: 10.1097/AUD.0000000000000647. PMID: 30134352
Johnson LC, Toro M, Vishnja E, Berish A, Mills B, Lu Z, Lieberman E
Hosp Pediatr 2018 Mar;8(3):141-147. doi: 10.1542/hpeds.2017-0060. PMID: 29472244
Cebulla M, Hofmann S, Shehata-Dieler W
Int J Pediatr Otorhinolaryngol 2014 May;78(5):756-61. Epub 2014 Feb 9 doi: 10.1016/j.ijporl.2014.02.003. PMID: 24642417

Recent clinical studies

Etiology

Madrid AM, Walker KA, Smith SB, Hood LJ, Prieve BA
Hear Res 2021 Aug;407:108277. Epub 2021 May 23 doi: 10.1016/j.heares.2021.108277. PMID: 34091212
Ankmnal-Veeranna S, Allan C, Allen P
J Am Acad Audiol 2019 Nov/Dec;30(10):904-917. Epub 2019 Jun 24 doi: 10.3766/jaaa.18046. PMID: 31241448
Hamed SA
Expert Opin Drug Saf 2017 Nov;16(11):1281-1294. Epub 2017 Aug 29 doi: 10.1080/14740338.2017.1372420. PMID: 28838247
Lammers MJ, van Eijl RH, van Zanten GA, Versnel H, Grolman W
J Assoc Res Otolaryngol 2015 Oct;16(5):669-78. Epub 2015 Jul 11 doi: 10.1007/s10162-015-0532-x. PMID: 26162414Free PMC Article
Li ZH, Chen C, Wilkinson AR, Jiang ZD
J Matern Fetal Neonatal Med 2011 Mar;24(3):536-40. Epub 2010 Jul 12 doi: 10.3109/14767058.2010.501126. PMID: 20624012

Diagnosis

Borenstein-Levin L, Taha R, Riskin A, Hafner H, Cohen-Vaizer A, Gordin A, Littner Y, Dinur G, Hochwald O, Kugelman A
Pediatr Res 2022 Jul;92(1):168-173. Epub 2021 Nov 17 doi: 10.1038/s41390-021-01849-0. PMID: 34789841
Trudeau S, Anne S, Otteson T, Hopkins B, Georgopoulos R, Wentland C
Am J Otolaryngol 2021 May-Jun;42(3):102923. Epub 2021 Jan 13 doi: 10.1016/j.amjoto.2021.102923. PMID: 33486206
Natale F, De Curtis M, Bizzarri B, Orlando MP, Ralli M, Liuzzi G, Caravale B, Franco F, Gaeta A, Giancotti A, Russo FY, Turchetta R
Ital J Pediatr 2020 Jan 6;46(1):3. doi: 10.1186/s13052-019-0767-y. PMID: 31906974Free PMC Article
Findlen UM, Hounam GM, Alexy E, Adunka OF
Ear Hear 2019 May/Jun;40(3):651-658. doi: 10.1097/AUD.0000000000000647. PMID: 30134352
Ramkissoon I, Batavia M
J Am Acad Audiol 2018 Sep;29(8):685-695. doi: 10.3766/jaaa.16161. PMID: 30222539

Therapy

Ramkissoon I, Batavia M
J Am Acad Audiol 2018 Sep;29(8):685-695. doi: 10.3766/jaaa.16161. PMID: 30222539
Silver MK, Shao J, Ji C, Zhu B, Xu L, Li M, Chen M, Xia Y, Kaciroti N, Lozoff B, Meeker JD
Int J Hyg Environ Health 2018 Apr;221(3):469-478. doi: 10.1016/j.ijheh.2018.01.010. PMID: 29402694Free PMC Article
Hamed SA
Expert Opin Drug Saf 2017 Nov;16(11):1281-1294. Epub 2017 Aug 29 doi: 10.1080/14740338.2017.1372420. PMID: 28838247
Indora V, Khaliq F, Vaney N
Int J Occup Environ Med 2017 Apr;8(2):109-116. doi: 10.15171/ijoem.2017.913. PMID: 28432372Free PMC Article
Li ZH, Chen C, Wilkinson AR, Jiang ZD
J Matern Fetal Neonatal Med 2011 Mar;24(3):536-40. Epub 2010 Jul 12 doi: 10.3109/14767058.2010.501126. PMID: 20624012

Prognosis

Madrid AM, Walker KA, Smith SB, Hood LJ, Prieve BA
Hear Res 2021 Aug;407:108277. Epub 2021 May 23 doi: 10.1016/j.heares.2021.108277. PMID: 34091212
Trudeau S, Anne S, Otteson T, Hopkins B, Georgopoulos R, Wentland C
Am J Otolaryngol 2021 May-Jun;42(3):102923. Epub 2021 Jan 13 doi: 10.1016/j.amjoto.2021.102923. PMID: 33486206
Lucchetti F, Nonclercq A, Avan P, Giraudet F, Fan X, Deltenre P
Hear Res 2021 Mar 1;401:108157. Epub 2020 Dec 17 doi: 10.1016/j.heares.2020.108157. PMID: 33360182
Lammers MJ, van Eijl RH, van Zanten GA, Versnel H, Grolman W
J Assoc Res Otolaryngol 2015 Oct;16(5):669-78. Epub 2015 Jul 11 doi: 10.1007/s10162-015-0532-x. PMID: 26162414Free PMC Article
Kochar DK, Kumawat BL, Halwai M, Kochar SK, Shubhakaran, Thanvi I
J Assoc Physicians India 2000 Mar;48(3):295-300. PMID: 11229113

Clinical prediction guides

Madrid AM, Walker KA, Smith SB, Hood LJ, Prieve BA
Hear Res 2021 Aug;407:108277. Epub 2021 May 23 doi: 10.1016/j.heares.2021.108277. PMID: 34091212
Ankmnal-Veeranna S, Allan C, Allen P
J Am Acad Audiol 2019 Nov/Dec;30(10):904-917. Epub 2019 Jun 24 doi: 10.3766/jaaa.18046. PMID: 31241448
Silver MK, Shao J, Ji C, Zhu B, Xu L, Li M, Chen M, Xia Y, Kaciroti N, Lozoff B, Meeker JD
Int J Hyg Environ Health 2018 Apr;221(3):469-478. doi: 10.1016/j.ijheh.2018.01.010. PMID: 29402694Free PMC Article
Indora V, Khaliq F, Vaney N
Int J Occup Environ Med 2017 Apr;8(2):109-116. doi: 10.15171/ijoem.2017.913. PMID: 28432372Free PMC Article
Lammers MJ, van Eijl RH, van Zanten GA, Versnel H, Grolman W
J Assoc Res Otolaryngol 2015 Oct;16(5):669-78. Epub 2015 Jul 11 doi: 10.1007/s10162-015-0532-x. PMID: 26162414Free PMC Article

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