Vol.:(0123456789) 1 3
European Archives of Oto-Rhino-Laryngology
https://doi.org/10.1007/s00405-018-5216-9
RHINOLOGY
Olfactory function and olfactory bulb volume in Wilson’s disease
Nazan Degirmenci
1
· Bayram Veyseller
2
· Hasmet Hanagasi
3
· Basar Bilgic
3
· Defne Gurbuz
4
· Ali Toprak
5
·
Orhan Ozturan
1
Received: 25 October 2018 / Accepted: 16 November 2018
© Springer-Verlag GmbH Germany, part of Springer Nature 2018
Abstract
Purpose To evaluate the olfactory function and the olfactory bulb (OB) volume changes in Wilson’s Disease (WD) patients.
Methods A prospective, controlled, single-blinded study was planned. 12 patients with WD (Group 1) and 12 healthy sub-
jects (Group 2) were included in the study. Connecticut Chemosensory Clinical Research Center (CCCRC) test was applied
to evaluate olfactory functions. OB volumes were measured with a 1.5 T General Electric Signa Excite MRI scanner.
Results There was a significant difference between the CCCRC scores of the two groups (p < 0.05). The difference of the
OB volumes of the two groups was insignificant (p > 0.05).
Conclusions WD patients are likely to experience olfactory dysfunction, so its assessment may be a useful tool to the follow-
up care of these patients, although further studies are needed to evaluate correlations in WD evolution.
Keywords Wilson’s disease · Olfactory dysfunction · Olfactory bulb · Olfaction
Introduction
Wilson’s disease (WD) is a rare autosomal recessive disorder
characterized by excessive copper deposition in the body,
mainly in the liver and the brain [1]. The diagnosis is usu-
ally based on the clinical and laboratory findings, including
Kayser–Fleischer rings, neurological symptoms, low serum
ceruloplasmin, increased urine and hepatic copper level
[2]. There are many reports on sensory deficits in primary
neurodegenerative disorders. Recent studies suggests that
the olfactory deficit in Parkinson Disease is progressive and
could correlate with other clinical markers [3]. This study
was conducted to evaluate the olfactory function and the
olfactory bulb (OB) volume changes in WD patients.
Materials and methods
Study design
A prospective, controlled, single-blinded study was planned.
* Nazan Degirmenci
nzndegirmenci@hotmail.com
Bayram Veyseller
bayveyseller@hotmail.com
Hasmet Hanagasi
hasmet@yahoo.com
Basar Bilgic
bilgicb@gmail.com
Defne Gurbuz
defnegurbuz@yahoo.com
Ali Toprak
atoprak@bezmialem.edu.tr
Orhan Ozturan
orhanent@yahoo.com
1
Department of Otorhinolaryngology and Head and Neck
Surgery, Bezmialem Vakif University, Istanbul, Turkey
2
Department of Otorhinolaryngology and Head and Neck
Surgery, Medical Faculty, Acibadem University, Istanbul,
Turkey
3
Department of Neurology, Istanbul Faculty of Medicine,
Istanbul University, Istanbul, Turkey
4
Department of Radiology, Okmeydani Training and Research
Hospital, Istanbul, Turkey
5
Department of Biostatistics and Medical Informatics,
Bezmialem Vakif University, Istanbul, Turkey