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High-frequency hearing impairment

MedGen UID:
42358
Concept ID:
C0018780
Disease or Syndrome
Synonyms: Hearing Loss, High Frequency; Hearing Loss, High-Frequency; High-Frequency Hearing Loss
 
HPO: HP:0005101

Definition

A type of hearing impairment affecting primarily the higher frequencies of sound (3,000 to 6,000 Hz). [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • High-frequency hearing impairment

Conditions with this feature

Pettigrew syndrome
MedGen UID:
162924
Concept ID:
C0796254
Disease or Syndrome
X-linked Dandy-Walker malformation with intellectual disability, basal ganglia disease and seizures (XDIBS), or Pettigrew syndrome is a central nervous system malformation characterized by severe intellectual deficit, early hypotonia with progression to spasticity and contractures, choreoathetosis, seizures, dysmorphic face (long face with prominent forehead), and brain imaging abnormalities such as Dandy-Walker malformation, and iron deposition. (From Mondo:0010574)
Progeroid short stature with pigmented nevi
MedGen UID:
224702
Concept ID:
C1261128
Disease or Syndrome
Mulvihill-Smith syndrome is characterized by premature aging, multiple pigmented nevi, lack of facial subcutaneous fat, microcephaly, short stature, sensorineural hearing loss, and mental retardation. Immunodeficiency may also be a feature. Adult manifestations include the development of tumors, a sleep disorder with severe insomnia, and cognitive decline (summary by Yagihashi et al., 2009).
Autosomal dominant nonsyndromic hearing loss 7
MedGen UID:
318614
Concept ID:
C1832379
Disease or Syndrome
Autosomal dominant deafness-7 (DFNA7) is a form of progressive sensorineural hearing loss with highly variable age at onset and severity, even within families. The age at onset ranges from congenital to mid-adulthood. Some patients may have associated vertigo (summary by Wesdorp et al., 2018).
Hearing loss, X-linked 4
MedGen UID:
376307
Concept ID:
C1848204
Disease or Syndrome
X-linked deafness-4 is a nonsyndromic form of progressive hearing loss with postlingual onset. Affected males show earlier onset of hearing loss than affected females (summary by del Castillo et al., 1996).
Deafness, autosomal dominant 39, with dentinogenesis imperfecta 1
MedGen UID:
340145
Concept ID:
C1854146
Disease or Syndrome
Autosomal dominant nonsyndromic hearing loss 17
MedGen UID:
350942
Concept ID:
C1863659
Disease or Syndrome
Any autosomal dominant nonsyndromic deafness in which the cause of the disease is a mutation in the MYH9 gene.
Autosomal dominant nonsyndromic hearing loss 2B
MedGen UID:
390742
Concept ID:
C2675236
Disease or Syndrome
Any autosomal dominant nonsyndromic deafness in which the cause of the disease is a mutation in the GJB3 gene.
Retinitis pigmentosa, X-linked, and sinorespiratory infections, with or without deafness
MedGen UID:
440716
Concept ID:
C2749137
Disease or Syndrome
X-linked retinitis pigmentosa and sinorespiratory infections with or without deafness (RPSRDF) is characterized by typical features of RP, including night blindness, constricted visual fields, progressive reduction in visual acuity, bone-spicule pigmentation, and extinguished responses on electroretinography. Affected individuals also experience severe recurrent sinorespiratory infections, and some develop progressive hearing loss. Carrier females may show an attenuated ocular and/or respiratory phenotype (Zito et al., 2003; Moore et al., 2006).
Combined oxidative phosphorylation deficiency 55
MedGen UID:
1806598
Concept ID:
C5676915
Disease or Syndrome
Combined oxidative phosphorylation deficiency-55 (COXPD55) is characterized by global developmental delay, hypotonia, short stature, and impaired intellectual development with speech disabilities in childhood. Indolent progressive external ophthalmoplegia phenotype has been described in 1 patient (summary by Olahova et al., 2021). For a discussion of genetic heterogeneity of combined oxidative phosphorylation deficiency, see COXPD1 (609060).

Professional guidelines

PubMed

Chan SL, Ng LS, Goh X, Siow CH, Goh HL, Goh BC, Cheo T, Loh KS, Brunham LR
Head Neck 2018 Jul;40(7):1425-1433. Epub 2018 Feb 16 doi: 10.1002/hed.25112. PMID: 29451951

Recent clinical studies

Etiology

Kasemsuk N, Chayopasakul V, Banhiran W, Prakairungthong S, Rungmanee S, Suvarnsit K, Atipas S, Keskool P
Otolaryngol Head Neck Surg 2023 Aug;169(2):201-209. Epub 2023 Jan 29 doi: 10.1177/01945998221120777. PMID: 36040818
Cho S, Park WJ, Ahn JS, Lim DY, Kim SH, Moon JD
Arch Environ Occup Health 2023;78(2):108-117. Epub 2022 Jul 14 doi: 10.1080/19338244.2022.2094306. PMID: 35833486
Mishra SK, Saxena U, Rodrigo H
Ear Hear 2022 May/Jun;43(3):822-835. doi: 10.1097/AUD.0000000000001140. PMID: 34700326
Michaud DS, Marro L, McNamee JP
J Acoust Soc Am 2021 Aug;150(2):1001. doi: 10.1121/10.0005589. PMID: 34470330
van Trier DC, van Nierop J, Draaisma JMT, van der Burgt I, Kunst H, Croonen EA, Admiraal RJC
Int J Pediatr Otorhinolaryngol 2015 Jun;79(6):874-878. Epub 2015 Apr 1 doi: 10.1016/j.ijporl.2015.03.021. PMID: 25862627

Diagnosis

Miyake H, Michikawa T, Nagahama S, Asakura K, Nishiwaki Y
Int J Environ Res Public Health 2022 Sep 28;19(19) doi: 10.3390/ijerph191912368. PMID: 36231667Free PMC Article
Michaud DS, Marro L, McNamee JP
J Acoust Soc Am 2021 Aug;150(2):1001. doi: 10.1121/10.0005589. PMID: 34470330
Smits JJ, Oostrik J, Beynon AJ, Kant SG, de Koning Gans PAM, Rotteveel LJC, Klein Wassink-Ruiter JS, Free RH, Maas SM, van de Kamp J, Merkus P; DOOFNL Consortium, Koole W, Feenstra I, Admiraal RJC, Lanting CP, Schraders M, Yntema HG, Pennings RJE, Kremer H
Hum Genet 2019 Jan;138(1):61-72. Epub 2018 Dec 8 doi: 10.1007/s00439-018-1965-1. PMID: 30535804Free PMC Article
Nagahama S, Kashino I, Hu H, Nanri A, Kurotani K, Kuwahara K, Dan M, Michikawa T, Akter S, Mizoue T, Murakami Y, Nishiwaki Y
BMJ Open 2018 Sep 17;8(9):e023220. doi: 10.1136/bmjopen-2018-023220. PMID: 30224397Free PMC Article
Zhang M
Acta Otolaryngol 2012 Sep;132(9):967-73. Epub 2012 Jun 5 doi: 10.3109/00016489.2012.679688. PMID: 22667466

Therapy

Wang Y, Yao X, Wang D, Ye C, Xu L
BMC Public Health 2024 Apr 25;24(1):1160. doi: 10.1186/s12889-024-18636-1. PMID: 38664666Free PMC Article
Cho S, Park WJ, Ahn JS, Lim DY, Kim SH, Moon JD
Arch Environ Occup Health 2023;78(2):108-117. Epub 2022 Jul 14 doi: 10.1080/19338244.2022.2094306. PMID: 35833486
Honeth L, Ström P, Ploner A, Bagger-Sjöbäck D, Rosenhall U, Nyrén O
Noise Health 2015 Sep-Oct;17(78):273-81. doi: 10.4103/1463-1741.165043. PMID: 26356369Free PMC Article
Fooladi MM
J Environ Public Health 2012;2012:235618. Epub 2011 Oct 13 doi: 10.1155/2012/235618. PMID: 22013454Free PMC Article
Gantz BJ, Turner CW
Laryngoscope 2003 Oct;113(10):1726-30. doi: 10.1097/00005537-200310000-00012. PMID: 14520097

Prognosis

Braffett BH, Lorenzi GM, Cowie CC, Gao X, Bainbridge KE, Cruickshanks KJ, Kramer JR, Gubitosi-Klug RA, Larkin ME, Barnie A, Lachin JM, Schade DS; DCCT/EDIC Research Group
Endocr Pract 2019 Dec;25(12):1243-1254. Epub 2019 Aug 14 doi: 10.4158/EP-2019-0193. PMID: 31412233Free PMC Article
Smits JJ, Oostrik J, Beynon AJ, Kant SG, de Koning Gans PAM, Rotteveel LJC, Klein Wassink-Ruiter JS, Free RH, Maas SM, van de Kamp J, Merkus P; DOOFNL Consortium, Koole W, Feenstra I, Admiraal RJC, Lanting CP, Schraders M, Yntema HG, Pennings RJE, Kremer H
Hum Genet 2019 Jan;138(1):61-72. Epub 2018 Dec 8 doi: 10.1007/s00439-018-1965-1. PMID: 30535804Free PMC Article
Nagahama S, Kashino I, Hu H, Nanri A, Kurotani K, Kuwahara K, Dan M, Michikawa T, Akter S, Mizoue T, Murakami Y, Nishiwaki Y
BMJ Open 2018 Sep 17;8(9):e023220. doi: 10.1136/bmjopen-2018-023220. PMID: 30224397Free PMC Article
Kytövuori L, Hannula S, Mäki-Torkko E, Sorri M, Majamaa K
Hear Res 2017 Nov;355:97-101. Epub 2017 Sep 28 doi: 10.1016/j.heares.2017.09.013. PMID: 28974383
Nakanishi N, Okamoto M, Nakamura K, Suzuki K, Tatara K
J Occup Environ Med 2000 Nov;42(11):1045-9. doi: 10.1097/00043764-200011000-00001. PMID: 11094781

Clinical prediction guides

Cho S, Park WJ, Ahn JS, Lim DY, Kim SH, Moon JD
Arch Environ Occup Health 2023;78(2):108-117. Epub 2022 Jul 14 doi: 10.1080/19338244.2022.2094306. PMID: 35833486
Mishra SK, Saxena U, Rodrigo H
Ear Hear 2022 May/Jun;43(3):822-835. doi: 10.1097/AUD.0000000000001140. PMID: 34700326
Braffett BH, Lorenzi GM, Cowie CC, Gao X, Bainbridge KE, Cruickshanks KJ, Kramer JR, Gubitosi-Klug RA, Larkin ME, Barnie A, Lachin JM, Schade DS; DCCT/EDIC Research Group
Endocr Pract 2019 Dec;25(12):1243-1254. Epub 2019 Aug 14 doi: 10.4158/EP-2019-0193. PMID: 31412233Free PMC Article
Smits JJ, Oostrik J, Beynon AJ, Kant SG, de Koning Gans PAM, Rotteveel LJC, Klein Wassink-Ruiter JS, Free RH, Maas SM, van de Kamp J, Merkus P; DOOFNL Consortium, Koole W, Feenstra I, Admiraal RJC, Lanting CP, Schraders M, Yntema HG, Pennings RJE, Kremer H
Hum Genet 2019 Jan;138(1):61-72. Epub 2018 Dec 8 doi: 10.1007/s00439-018-1965-1. PMID: 30535804Free PMC Article
Nagahama S, Kashino I, Hu H, Nanri A, Kurotani K, Kuwahara K, Dan M, Michikawa T, Akter S, Mizoue T, Murakami Y, Nishiwaki Y
BMJ Open 2018 Sep 17;8(9):e023220. doi: 10.1136/bmjopen-2018-023220. PMID: 30224397Free PMC Article

Recent systematic reviews

Kasemsuk N, Chayopasakul V, Banhiran W, Prakairungthong S, Rungmanee S, Suvarnsit K, Atipas S, Keskool P
Otolaryngol Head Neck Surg 2023 Aug;169(2):201-209. Epub 2023 Jan 29 doi: 10.1177/01945998221120777. PMID: 36040818

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