Bilgeç et al. Vestibulocochlear System in Pseudoexfoliation Syndrome DOI: 10.4274/tjo.galenos.2020.14892 Turk J Ophthalmol 2021;51:156-160 Ori gi nal Ar tic le Evaluation of the Vestibulocochlear System in Patients with Pseudoexfoliation Syndrome Mustafa Değer Bilgeç*, Nagehan Erdoğmuş Küçükcan**, Leman Birdane***, Armağan İncesulu****, Nilgün Yıldırım* *Eskişehir Osmangazi University Faculty of Medicine, Department of Ophthalmology, Eskişehir, Turkey **Çukurova Dr. Aşkım Tüfekçi State Hospital, Clinic of Otorhinolaryngology, Adana, Turkey ***Yunus Emre State Hospital, Clinic of Otorhinolaryngology, Eskişehir, Turkey ****Eskişehir Osmangazi University Faculty of Medicine, Department of Otorhinolaryngology, Eskişehir, Turkey Abstract Objectives: Patients with pseudoexfoliation syndrome (PES) can also have sensorineural hearing loss as well as balance problems. Our aim was to evaluate vestibulocochlear system involvement in PES patients. Materials and Methods: The study included 16 subjects with PES (study group) with a mean age of 66.12±5.64 years and 17 healthy subjects (control group) with a mean age of 61.70±8.46 years. Both groups underwent ophthalmological, neuro-otological, audiological, and vestibular evaluation. Pure-tone audiometry and tympanometry were performed as audiological tests and bithermal caloric test and vestibular-evoked myogenic potential (VEMP) testing were used as vestibular tests. The Romberg, tandem Romberg, and Unterberger tests were also performed. Results: In the PES group, bithermal caloric tests revealed right canal paresis in 6 patients, left canal paresis in 3 patients, and bilateral stimulation loss in 2 patients, despite no clinical evidence of balance loss. Paresis was not detected in any of the control subjects. Unilateral VEMP responses could not be obtained in 3 patients in the PES group. The ocular PES patients whose VEMP waves were obtained differed significantly from the control group (p<0.05). In office tests for vestibular evaluation, pathologic findings were found in 7 of 16 patients in the study group and only 4 subjects in the control group. Audiological evaluation with pure-tone thresholds revealed sensorineural decline at 4000 and 8000 Hertz in the PES patients. A statistically significant difference was found between the study group and the control group (p<0.05). Conclusion: Patients with PES showed elevation in pure-tone thresholds and a decrease in superior and inferior vestibular nerve function, demonstrating that the vestibular system as well as the auditory system are affected in PES. Keywords: Pseudoexfoliation syndrome, vestibular diseases, vestibular function tests, vestibular evoked myogenic potentials Address for Correspondence: Mustafa Değer Bilgeç, Eskişehir Osmangazi University Faculty of Medicine, Department of Ophthalmology, Eskişehir, Turkey E-mail: [email protected] ORCID-ID: orcid.org/0000-0002-9972-2147 Received: 18.11.2019 Accepted: 27.07.2020 Cite this article as: Bilgeç MD, Erdoğmuş Küçükcan N, Birdane L, İncesulu A, Yıldırım N. Evaluation of the Vestibulocochlear System in Patients with Pseudoexfoliation Syndrome. Turk J Ophthalmol 2021;51:156-160 ©Copyright 2021 by Turkish Ophthalmological Association Turkish Journal of Ophthalmology, published by Galenos Publishing House. 156 Bilgeç et al. Vestibulocochlear System in Pseudoexfoliation Syndrome Introduction audiological, and vestibular evaluations were performed in both groups. Ophthalmological examination for all patients consisted Pseudoexfoliation syndrome (PES), first described by 1 of corrected visual acuity, biomicroscopy, intraocular pressure Lindberg in 1917, is a systemic disorder characterized by measurement, and fundus examination. excessive synthesis and accumulation of fibrillary material in the Vestibular evaluation was performed in both groups using ocular and extraocular tissues. This pseudoexfoliative material is vestibular-evoked myogenic potential (VEMP) testing, bithermal believed to have many sources and occur secondary to abnormal caloric test, and office vestibular tests; audiological evaluation basement membrane constituents produced by aging epithelial 2 was performed using pure-tone audiometry and tympanometric cells. examination. The Romberg, tandem Romberg, Unterberger, Pseudoexfoliation fibrils can accumulate in the anterior and Dix-Hallpike tests were performed as office vestibular segment structures of the eye such as the conjunctiva, corneal tests. Video electronystagmography, another vestibular test, endothelium, anterior lens capsule, trabeculum, iris, zonules, was not used because it does not give reliable results in ocular and ciliary body, leading to glaucoma and subsequent progressive pathologies. vision loss.3 Light and electron microscopic analyses and immunohistochemical methods have also demonstrated the VEMP Test presence and accumulation of pseudoexfoliative material in The cervical VEMP test is based on measuring the the inner ear, as well as the extracellular matrices of systemic electromyographic activity of the sternocleidomastoid muscle organs such as the skin, heart, lung, and liver.4,5 In addition, the in response to high-intensity acoustic stimuli to the saccular detection of material accumulation in the connective tissue layers macula. It is generally used to determine whether the saccule, of the skin and internal organs, the periphery of blood vessels, inferior vestibular nerve, and central connections are working the smooth and striated muscle layers of the internal organs, normally. The VEMP pathway is formed by the saccule, and the cardiac muscle indicates that PES is a multisystemic inferior vestibular nerve, lateral vestibular nucleus, medial disorder rather than just an ocular disease.6,7 Its association with vestibulospinal pathway, and sternocleidomastoid muscle after diseases that cause high mortality and morbidity, particularly auditory stimulation of the saccule. VEMP tracings were cardiovascular and cerebrovascular diseases, further increases the recorded with a Medelec Synergy device. The first positive wave importance of PES.8 was taken as p13 and the first negative wave as n23. The p13 The decline in hearing in PES patients at advanced ages has and n23 latencies and the amplitude between the two waves been attributed to the fibrillary material accumulated in the (p13-n23) were measured. The latency and amplitude values of cochlea preventing the conversion of sound energy to electrical the patient group were compared with normative data obtained energy.9,10,11 Several studies have shown that pseudoexfoliation from the control group.15 fibrils accumulate in the basilar membrane, tectorial membrane, Bithermal Caloric Test and stria vascularis of the inner ear. Embryologically, the Bitermal caloric test recordings were performed using a ocular anterior segment and inner ear originate from the same CHARTR water caloric stimulator, model NCI-480. Subjects germ layer.12 However, there are few studies in the literature were placed in supine position with the head at 30° anteroflexion demonstrating the involvement of the vestibular apparatus, during the test. The test was performed by administering water which constitutes another part of the inner ear.13 at two temperatures, 7°C above and below body temperature Balance is provided mainly by the visual, vestibular, and (30/44°C), to the external auditory canal. Activation is measured proprioceptive systems. The central nervous system integrates in the lateral semicircular canal, which is the most superficial information from the relevant peripheral organs, then maintains and easiest to reach. This test can also provide information about balance via the necessary reflexes.14 the superior vestibular nerve. The test results were evaluated In this study, we planned to investigate whether PES also in terms of canal paresis and directional superiority. A 25% affects the balance system to the same degree as the auditory difference in the durations of nystagmus occurring with hot and system due to the likely accumulation of pseudoexfoliation cold stimuli in both canals was regarded as canal paresis.16 fibrils. Romberg Test Materials and Methods While standing with feet together, head upright, and arms at their sides, the subject was asked to close their eyes. After thus A prospective, case-controlled study was conducted. eliminating the auxiliary role of vision in balance, the subject’s Approval was obtained from the Eskişehir Osmangazi University balance is observed and any vestibular system disorders are Ethics Committee. Informed consent forms were obtained from revealed. Movement in small circles suggested a central origin, the patients. while tilting backward or to the side was considered more Sixteen individuals with ocular PES (study group) and 17 suggestive of a cerebellar disorder.16 healthy subjects (control group) were included. Of the 16 patients in the study group, 10 were male and 6 Unterberger Test were female. Of the 17 healthy volunteers in the control group, Subjects were asked to take 40-50 steps in place with their 12 were male and 5 were female. Ophthalmological, neurological, hands outstretched in front of them. Rotational deviation 157 Turk J Ophthalmol 51; 3: 2021 to either side that did not disappear with repeated tests was VEMP testing was performed in all 16 PES patients, but interpreted as indicating vestibular pathology on that side.16 unilateral VEMP waves could not be obtained in 3 patients. Dix-Hallpike Test The p13, n23, and amplitude values of the 29 ears with