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Auditory Deficits in Neurological Disorders Ubytki Słuchu W Chorobach Neurologicznych

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Auditory Deficits in Neurological Disorders Ubytki Słuchu W Chorobach Neurologicznych ARtykuł ORYGINALNY / ORIGINAL ARTICLE Auditory deficits in neurological disorders Ubytki słuchu w chorobach neurologicznych 1DBAE 2BCE 3BC 4BDF Authors’ Contribution: Tomasz Przewoźny , Anna Gójska-Grymajło , Tomasz Szmuda , Karolina Markiet A – Study Design B – Data Collection 1 C – Statistical Analysis Department of Otolaryngology, Medical University of Gdańsk, Smoluchowskiego 17, 80-214 Gdańsk, Poland D – Data Interpretation 2 E – Manuscript Preparation Department of Neurology of Adults, Medical University of Gdańsk, Dębinki 7, 80-211 Gdańsk, Poland F – Literature Search 3Department of Neurosurgery, Medical University of Gdańsk, Smoluchowskiego 17, 80-214 Gdańsk, Poland G – Funds Collection 4II Department of Radiology, Medical University of Gdańsk, Smoluchowskiego 17, 80-214 Gdańsk, Poland Article history: Received: 04.08.2015 Accepted: 16.08.2015 Published: 30.10.2015 ABSTRACT: Neurological diseases present with diverse and often complex symptomatology. Focal neurological signs such as pa- resis, aphasia or visual field deficits together with often serious general state of a neurological patient usually push auditory symptoms into the background. Here, we present a review of literature on central and peripheral auditory disturbances that can appear in the course of most common neurological diseases. We present: cerebral stroke, co- chleovestibular nerve compression syndrome, cerebral palsy, multiple sclerosis, epilepsy, myasthenia gravis and brain tumors. We focus on the neuroanatomical basis of auditory dysfunctions, their character and prevalence typical for the abovementioned diseases. Theoretical considerations are supported by broad audiological and neuroimaging studies of our patients. Auditory symptoms in neurological diseases seem to be rare. However, knowledge of these symptoms and their origin can be helpful in proper diagnosis and comprehensive patient management. KEYWORDS: multiple sclerosis, ischemic cerebral stroke, epilepsy, sensorineural hearing loss, sudden deafness, tinnitus STRESZCZENIE: Choroby neurologiczne objawiają się w sposób różnorodny i złożony. Ogniskowe objawy neurologiczne, takie jak niedowłady, afazja, ubytki w polu widzenia, którym bardzo często towarzyszy pogorszenie stanu pacjenta sprawiają, że objawy słuchowe schodzą na drugi plan. W niniejszym artykule przedstawiamy przegląd literatury dotyczącej ośrodkowych oraz obwodowych zaburzeń słuchu w przebiegu najczęstszych chorób neurologicznych. Opisujemy udar mózgu, konflikt naczyniowo-nerwowy nerwu przedsionkowo-ślimakowego, porażenie mózgowe, stwardnienie rozsiane, padaczkę, miastenię oraz guzy mózgu. Zwracamy uwagę na neuroanatomiczne podstawy zaburzeń słuchu, ich charakter oraz częstość występowania w wymienionych wyżej chorobach. Rozważania teoretyczne uzupełnione zostały prezentacją wyników licznych badań audiologicznych i neuroobrazowych wykonanych u naszych pacjentów. Objawy słuchowe w chorobach neurologicznych wydają się rzadkie, jednakże wiedza dotycząca ich pochodzenia może być pomocna w postawieniu właściwej diagnozy i zastosowaniu kompleksowego leczenia SŁOWA KLUCZOWE: stwardnienie rozsiane, udar niedokrwienny mózgu, padaczka, niedosłuch odbiorczy, nagła głuchota, szum uszny INTRODUCTION ogy to support diagnostic and therapeutic decisions in differ- ent clinical fields. The usefulness of these methods in certain Neurological diseases have complex symptomatology, with au- diseases, especially neurological ones, is regarded comparable diological symptoms included. However, audiological signs are to neuroimaging studies. often neglected by neurologists and otolaryngologists, which may result in a late or wrong diagnosis. In the last 30 years Audiological symptoms of neurological diseases are com- there has been substantial progress in the development of the bined with pathology of different levels of the auditory system, auditory diagnostics based on the electrophysiological mod- from the middle ear to the cerebral cortex. They include more els. These diagnostics are commonly used in modern audiol- commonly diagnosed - hearing loss or tinnitus [1, 2] togeth- - - - - - OTOLARYNGOLOGIA POLSKA, TOM 69, NR 5 (2015), P. 29-43 DOI: 10.5604/00306657.1170416 29 ARtykuł ORYGINALNY / ORIGINAL ARTICLE er with less often found higher auditory dysfunctions such as between these two groups. Other authors confirmed correla- the impairment of understanding speech, sound localization tions between ischemic cerebral processes and hearing loss [1, disability, hearing hypersensitivity, phonophobia or auditory 2, 10, 11]. Sudden hearing loss in cerebral stroke may be com- hallucinations [3-6]. plete and it can affect 1.4 to 21% patients, depending on the inclusion/exclusion criteria of the performed studies [1, 2, 10, The aim of the following article was to present audiological 11]. Huang et al. [12] in a study on 503 stroke patients, found symptoms typical for common neurological diseases: cerebral sudden bilateral hearing loss in 7 (1.4%) subjects. Yamasaba et stroke, cochleovestibular nerve compression syndrome, cer- al. [10] found sudden hearing loss and vertigo in 15 (21%) of ebral palsy, multiple sclerosis, epilepsy, myasthenia, cerebel- 70 patients with transient ischemic attack in the vertebrobasi- lopontine angle tumors and cerebral tumors. The authors, on lar territory. Lee et al. [11] described 16 patients with AICA the basis of available literature, present the type, prevalence stroke - 5 (31%) had acute hearing symptoms (hearing loss and pathological background of the auditory dysfunctions in and vertigo). Interestingly, the symptoms appeared 1-10 days the abovementioned diseases. before the onset of other brainstem and cerebellar symptoms. The authors distinguished two acute hearing syndromes: 1) recurrent, transient hearing loss with or without tinnitus that CEREBROVASCULAR DISEASES lasted for several days; and 2) single, prolonged hearing loss with or without tinnitus. The most commonly affected brain Cerebral stroke area in this study was the middle cerebellar peduncle. In four Stroke can affect the territory of the main cerebral arteries: of the five patients hearing loss of variable degree was found the internal carotid artery and its two branches – the middle and all the patients presented vestibular damage in the caloric cerebral artery and the anterior cerebral artery, and of the pos- testing on the side of the ischemic lesion. terior cerebral arteries and the basilar artery. Cerebral tissue ischemia begins 25 seconds after vessel closure and leads to irreversible tissue and metabolic damage after 4-5 minutes. It is the territory of the middle cerebral artery where the vascular incidents are most common. The internal ear and the vestib- ulocochlear nerve are vascularized by the labyrinthine artery, a branch of the anterior inferior cerebellar artery (AICA) (in 60% of cases) or less often of the basilar artery (40% of cases). The internal ear is particularly susceptible to ischemia due to its high metabolic rate and lack of collateral circulation [7]. On the contrary, the vestibulocochlear nerve (the VIII cranial nerve) has an additional collateral circulation. According to WHO the cerebrovascular diseases are divid- ed into the ischemic and hemorrhagic stroke, and the sub- arachnoidal bleeding, where 80% of the incidents are due to the ischemic stroke. Auditory symptoms such as the hearing loss, tinnitus and vertigo appear in the posterior cerebral ar- tery strokes. First description of these symptoms was given by Adams in 1943 [8] – vertigo, hearing loss, facial nerve pal- sy, ataxia, nystagmus and hypoesthesia were caused by AICA occlusion. Since then many clinical descriptions of the cere- bral stroke patients with auditory symptoms have appeared, Fig. 1. Horizontal minimum audible angle test (HMAAT) results and MRI however, most of them are case reports. Clinical analyses of findings in a 45-year-old patient with bilateral symmetric mild bigger groups of stroke patients with auditory symptoms are sensorineural hearing loss and poor speech discrimination on the seventh scarce [1, 2, 9, 10], and the results are inconsistent, mainly due day after the incident of stroke. (A) Black line-95th percentile values for the age-matched subgroup of controls, red dotted line-results of the patient; to variable location of stroke lesions. Hariri et al. [9], who com- (incorrect result for all azimuths: 0°[9°]; 45° [24°]; 90° [21°]; 180°[24°]; pared hearing by pure tone audiometry (PTA) in 25 stroke pa- 225°[20°]; 270°[16.5°] and 315°[18°]. (B) Axial FLAIR image shows a diffuse, tients aged 58 to 85, with hearing of 25 healthy individuals of periventricular area of hyperintensity. Axial FLAIR image (C) and coronal T2-weighted image (D) show the extent of ischemic lesions. the same age, did not find statistically significant differences - - - - - 30 WWW.OTOLARYNGOLOGYPL.COM ARtykuł ORYGINALNY / ORIGINAL ARTICLE Fig. 2. Anatomical structures of the cerebellopontine angle. Neurovascular conflict between the VIII and VII cranial nerves and the anterior inferior cerebellar artery. Abbreviations: CN-cranial nerve, AICA-anterior inferior cerebellar artery, PICA-posterior inferior cerebellar artery, FL-flocculus, LA-labyrinthine artery, NI- intermediate nerve. In a comprehensive study by Lee-Baloh et al. [1], sudden hear- ing speech. Multiple crossings of the auditory pathways are ing loss was found in 29 subjects (8%) of 364 patients, and the the reason for a vascular lesion to cause both ipsilateral and hearing loss correlated mostly with the
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