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Deafness, sensorineural, with peripheral neuropathy and arterial disease

MedGen UID:
343766
Concept ID:
C1852280
Disease or Syndrome
Synonym: Deafness, Sensorineural, with Peripheral Neuropathy and Arterial Disease
 
Monarch Initiative: MONDO:0007425
OMIM®: 124950

Clinical features

From HPO
Retinal arteriolar constriction
MedGen UID:
853673
Concept ID:
C2176208
Finding
Decreased retinal arteriolar diameters, which may decrease blood flow and slow oxygen delivery to regions of the retina.
Peripheral arterial stenosis
MedGen UID:
870815
Concept ID:
C4025272
Disease or Syndrome
Narrowing of peripheral arteries with reduction of blood flow to the limbs. This feature may be quantified as an ankle-brachial index of less than 0.9, and may be manifested clinically as claudication.
Bilateral sensorineural hearing impairment
MedGen UID:
96788
Concept ID:
C0452138
Disease or Syndrome
A bilateral form of sensorineural hearing impairment.
Progressive hearing impairment
MedGen UID:
331224
Concept ID:
C1842138
Finding
A progressive form of hearing impairment.
Headache
MedGen UID:
9149
Concept ID:
C0018681
Sign or Symptom
Cephalgia, or pain sensed in various parts of the head, not confined to the area of distribution of any nerve.
Polyneuropathy
MedGen UID:
57502
Concept ID:
C0152025
Disease or Syndrome
A generalized disorder of peripheral nerves.
Increased CSF protein concentration
MedGen UID:
329971
Concept ID:
C1806780
Finding
Increased concentration of protein in the cerebrospinal fluid.
Skin rash
MedGen UID:
1830322
Concept ID:
C5779628
Sign or Symptom
A red eruption of the skin.
Concave nasal ridge
MedGen UID:
78105
Concept ID:
C0264169
Finding
Nasal ridge curving posteriorly to an imaginary line that connects the nasal root and tip.
Optic papillitis
MedGen UID:
10565
Concept ID:
C0030353
Finding
Papilledema refers to edema (swelling) of the optic disc secondary to any factor which increases cerebral spinal fluid pressure.

Recent clinical studies

Etiology

Gomes I, Girão A, Gomes J, Rebelo O, Jesus-Ribeiro J
Acta Neurol Belg 2022 Feb;122(1):123-128. Epub 2021 Apr 27 doi: 10.1007/s13760-021-01683-5. PMID: 33905106
Teton ZE, Holste KG, Hardaway FA, Burchiel KJ, Raslan AM
J Neurosurg 2020 Jan 1;132(1):232-238. Epub 2019 Jan 11 doi: 10.3171/2018.8.JNS18239. PMID: 30641844
Ferro JM, Oliveira SN, Correia L
Handb Clin Neurol 2014;120:595-605. doi: 10.1016/B978-0-7020-4087-0.00040-1. PMID: 24365340
Jatti K, Paisey R, More R
Eur J Hum Genet 2012 Jan;20(1):117-8. Epub 2011 Sep 7 doi: 10.1038/ejhg.2011.168. PMID: 21897446Free PMC Article
Makins AE, Nikolopoulos TP, Ludman C, O'Donoghue GM
Laryngoscope 1998 Nov;108(11 Pt 1):1739-42. doi: 10.1097/00005537-199811000-00027. PMID: 9818836

Diagnosis

Gomes I, Girão A, Gomes J, Rebelo O, Jesus-Ribeiro J
Acta Neurol Belg 2022 Feb;122(1):123-128. Epub 2021 Apr 27 doi: 10.1007/s13760-021-01683-5. PMID: 33905106
Parida P, Dubbudu A, Biswal SR, Sharawat IK, Panda PK
Brain Dev 2021 Feb;43(2):314-319. Epub 2020 Oct 20 doi: 10.1016/j.braindev.2020.09.009. PMID: 33092935
Choi SY, Kee HJ, Park JH, Kim HJ, Kim JS
J Vestib Res 2014;24(5-6):443-51. doi: 10.3233/VES-140524. PMID: 25564087
Ferro JM, Oliveira SN, Correia L
Handb Clin Neurol 2014;120:595-605. doi: 10.1016/B978-0-7020-4087-0.00040-1. PMID: 24365340
Allmendinger AM, Mallery RM, Magro CM, Wang N, Egan RA, Samuels MA, Callahan A, Viswanadhan N, Klufas RA, Hsu L, Prasad S
J Neurol Sci 2014 Feb 15;337(1-2):91-6. Epub 2013 Nov 22 doi: 10.1016/j.jns.2013.11.023. PMID: 24290499

Therapy

Parida P, Dubbudu A, Biswal SR, Sharawat IK, Panda PK
Brain Dev 2021 Feb;43(2):314-319. Epub 2020 Oct 20 doi: 10.1016/j.braindev.2020.09.009. PMID: 33092935
Teton ZE, Holste KG, Hardaway FA, Burchiel KJ, Raslan AM
J Neurosurg 2020 Jan 1;132(1):232-238. Epub 2019 Jan 11 doi: 10.3171/2018.8.JNS18239. PMID: 30641844
Aquilina K, Kumar R, Lu J, Rawluk D
Acta Neurochir (Wien) 2005 Mar;147(3):291-7; discussion 297. doi: 10.1007/s00701-004-0460-8. PMID: 15662570
Brey RL, Escalante A
Lupus 1998;7 Suppl 2:S67-74. doi: 10.1177/096120339800700216. PMID: 9814677

Prognosis

Turcotte EL, Patra DP, Abi-Aad KR, Welz ME, Weisskopf PA, Bendok BR
World Neurosurg 2020 Mar;135:233. Epub 2019 Dec 16 doi: 10.1016/j.wneu.2019.12.046. PMID: 31857267
van der Steenstraten F, de Ru JA, Witkamp TD
Ann Otol Rhinol Laryngol 2007 Apr;116(4):248-52. doi: 10.1177/000348940711600404. PMID: 17491521
Horii A, Okumura K, Kitahara T, Kubo T
Acta Otolaryngol 2006 Feb;126(2):170-3. doi: 10.1080/00016480500279977. PMID: 16428195

Clinical prediction guides

Gomes I, Girão A, Gomes J, Rebelo O, Jesus-Ribeiro J
Acta Neurol Belg 2022 Feb;122(1):123-128. Epub 2021 Apr 27 doi: 10.1007/s13760-021-01683-5. PMID: 33905106
Turcotte EL, Patra DP, Abi-Aad KR, Welz ME, Weisskopf PA, Bendok BR
World Neurosurg 2020 Mar;135:233. Epub 2019 Dec 16 doi: 10.1016/j.wneu.2019.12.046. PMID: 31857267
Clift JM, Wong RD, Carney GM, Stavinoha RC, Boyev KP
Ann Otol Rhinol Laryngol 2009 May;118(5):356-61. doi: 10.1177/000348940911800507. PMID: 19548385
Chadha NK, Weiner GM
Clin Otolaryngol 2008 Feb;33(1):5-11. doi: 10.1111/j.1749-4486.2007.01597.x. PMID: 18302544
Rosseau GL, Jannetta PJ, Hirsch B, Møller MB, Møller AR
Am J Otol 1993 Jul;14(4):392-7. PMID: 8238278

Recent systematic reviews

Chadha NK, Weiner GM
Clin Otolaryngol 2008 Feb;33(1):5-11. doi: 10.1111/j.1749-4486.2007.01597.x. PMID: 18302544

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