Evidence map›Paper›PMID 34689817›Full record

ArticleOrphanet journal of rare diseases2021

Hearing problems in patients with hereditary gelsolin amyloidosis.

Tuuli Mustonen, Ville Sivonen, Sari Atula, Sari Kiuru-Enari, Saku T Sinkkonen

Open access · goldAbstract read
PubMed Full text at PMC Publisher
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Article in Orphanet journal of rare diseases, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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0citing papers in PubMed
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1 · What the graph read from it

What it found

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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

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3 · Its place in the literature

Who cites it

0 citing papers in PubMed, 0 citations in OpenAlex.

No citing paper in PubMed yet.

4 · The record

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5 · Who and what money

Authors and funding

5 authors at 1 institution in 1 country.

Tuuli MustonenClinical Neurosciences, Neurology, Helsinki University Hospital and University of Helsinki, HYKS, Tornisairaala, Neupkl, Haartmaninkatu 4, 00029, Helsinki, Finland. tuuli.mustonen@helsinki.fi.ORCID 0000-0002-9551-3022
Ville SivonenDepartment of Otorhinolaryngology - Head and Neck Surgery, Head and Neck Center, Helsinki University Hospital and University of Helsinki, Helsinki, Finland.
Sari AtulaClinical Neurosciences, Neurology, Helsinki University Hospital and University of Helsinki, HYKS, Tornisairaala, Neupkl, Haartmaninkatu 4, 00029, Helsinki, Finland.
Sari Kiuru-EnariClinical Neurosciences, Neurology, Helsinki University Hospital and University of Helsinki, HYKS, Tornisairaala, Neupkl, Haartmaninkatu 4, 00029, Helsinki, Finland.
Saku T SinkkonenDepartment of Otorhinolaryngology - Head and Neck Surgery, Head and Neck Center, Helsinki University Hospital and University of Helsinki, Helsinki, Finland.
University of Helsinki · FI

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundGelsolin amyloidosis (AGel amyloidosis) is a hereditary form of systemic amyloidosis featuring ophthalmological, neurological and cutaneous symptoms. Previous studies based mainly on patients' self-reporting have indicated that hearing impairment might also be related to the disease, considering the progressive cranial neuropathy characteristic for AGel amyloidosis. In order to deepen the knowledge of possible AGel amyloidosis-related hearing problems, a clinical study consisting of the Speech, Spatial and Qualities of Hearing Scale (SSQ) questionnaire, clinical examination, automated pure-tone audiometry and a speech-in-noise test was designed.

resultsOf the total 46 patients included in the study, eighteen (39%) had self-reported hearing loss. The mean scores in the SSQ were 8.2, 8.3 and 8.6 for the Speech, Spatial and Qualities subscales, respectively. In audiometry, the mean pure tone average (PTA) was 17.1 (SD 12.2) and 17.1 (SD 12.3) dB HL for the right and left ears, respectively, with no difference to gender- and age-matched, otologically normal reference values. The average speech reception threshold in noise (SRT) was - 8.2 (SD 1.5) and - 8.0 (SD 1.7) dB SNR for the right and left ears, respectively, which did not differ from a control group with a comparable range in PTA thresholds.

conclusionAlthough a significant proportion of AGel amyloidosis patients experience subjective difficulties in hearing there seems to be no peripheral or central hearing impairment at least in patients up to the age of 60 years.

Indexed as

Amyloid Neuropathies, FamilialGelsolinAudiometry, Pure-ToneHearingHumansMiddle AgedNoiseGelsolinAutomated audiometryGelsolin amyloidosisHearing lossSpeech-in-noiseSpeech Spatial and Qualities of Hearing Scale

Identifiers

PMID34689817
PMCPMC8543933
OpenAlexW3209635302

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.