Neurol Sci (2008) 29:359–362 DOI 10.1007/s10072-008-0996-0 C A S E R E P O RT Recurrent audiovestibular disturbance initially mimicking Ménière’s disease in a patient with anterior inferior cerebellar infarction Jong-Ho Park • Hyeyun Kim • Hyun-Jeong Han Received: 13 February 2008 / Accepted in revised form: 10 July 2008 © Springer-Verlag 2008 Abstract An anterior inferior cerebellar artery (AICA) stroke is characterized by vertigo, tinnitus, and deafness in addition to facial weakness, hemiataxia, and hypalgesia. Sometimes, it can present as sudden deafness with vertigo, without brainstem or cerebellar signs. We report a 55-year-old woman with hypertension and diabetes, showing recurrent audiovestibular disturbance before a typical pattern of AICA infarction, which was initially diagnosed as Ménière’s disease. In elderly patients with recurrent hearing loss and vertigo lasting several minutes, lack classic brainstem or cerebellar signs, if they have vascular risk factors, physicians may also consider the potential symptom of AICA infarction. Keywords Recurrent deafness · Vertigo · Anterior inferior cerebellar artery infarction · Elderly · Risk factor Introduction Ménière’s disease (MD) is an inner ear disorder responsible for sensorineural hearing loss; it is associated with sudden vertigo, tinnitus, and ear fullness. Typical MD simultaneously accompanies hearing and vestibular dysfunctions, as described above [1]. MD is more prevalent in women than men; its attacks are sudden and may last from 20 minutes to several hours [2, 3]. Although MD most commonly affects people in the fifth decade of life [2, 4], it is not at all uncommon in the elderly [5]. The anterior inferior cerebellar artery (AICA) supplies the middle cerebellar peduncle, cerebellar flocculus, and inferolateral tegmentum of the pons. Therefore, AICA occlusion is associated with ipsilateral facial palsy and sensory impairment, ataxia, and Horner’s sign, in addition to hearing and vestibular dysfunctions [6]. However, AICA infarction may also present with sudden hearing loss with vertigo, without obvious neurological deficits [7–10]. We report a patient with another clinical pattern of AICA infarction, which initially mimicked MD. Case report J.H. Park (쾷) · H. Kim · H.J. Han Department of Neurology Myongji Hospital, Kwandong University College of Medicine 697-24, Hwajeong-dong, Deokyang-gu, Goyang-si Kyunggi-do 412-270, Republic of Korea e-mail: neurocraft@kd.ac.kr A 55-year-old woman had suffered from hypertension diagnosed seven years earlier; she had also been diagnosed with non-insulin dependent diabetes mellitus and hyperthyroidism five years earlier. She had received a thyroidectomy and was taking antihypertensive, oral hypoglycaemic, and thyroid medications. One month before her admission, she suddenly developed vertigo, reduction of hearing in the right ear, and tinnitus (characterized by intermittent, nonpulsatile noise) for several minutes during normal routine activity. Seven and five days before her admission, she experienced such recurrent symptoms for 2~3 h, respectively. She had no 360 Neurol Sci (2008) 29:359–362 other accompanying neurological symptoms, except the aforementioned audiovestibular symptoms. She visited the local emergency room and was diagnosed with peripheral vertigo without being given further evaluation. The audiovestibular symptoms started suddenly and ameliorated gradually in the few days that followed. Following these attacks, she complained of residual hypoacusis and vertigo for a few days. Two days before her admission, she again developed the aforementioned symptoms, which were more intense and lasted continuously. There was no prior history of head trauma, ear disease, syphilis, or medication with ototoxic drugs. When she was first examined on admission day, her vital signs included a blood pressure of 130/80 mmHg, a pulse rate of 80 bpm, a respiration rate of 20/min, and a body temperature of 36.5°C. An otological examination revealed normal tympanic membranes. On neurological examination, she had spontaneous, leftbeating nystagmus with horizontal and torsional components in primary position and on gaze to the left. She had no dysarthria, facial/limb weakness, sensory loss, or ataxia. Weber and the Rinne tests demonstrated a sensorineural hearing defect on the right side. Pure tone audiometry depicted ipsilateral sensorineural hearing defect (Fig. 1). Under a tentative diagnosis of probable MD, she started to take a low-salt diet and diuretics (dichlozide 25 mg/day) with symptomatic therapy. On the second admission day, right facial palsy developed. During her neurological examination, right side upper and lower limb ataxia and partial Horner’s sign (ptosis, miosis, and hypohydrosis) were noted, in addition to ipsilateral facial hypaesthesia and peripheral type palsy. Brain diffusion-weighted image (DWI) showed hyperintensities on the right middle cerebellar peduncle and pontine tegmentum (Fig. 2a). She was immediately transferred to the department of neurology and was treated with antiplatelet medication (clopidogrel 75 mg/day). There was no definite stenotic change to the vertebrobasilar system on 3-D time-of-flight MR angiography. Brainstem auditory evoked response (BAER) revealed no Fig. 1 Pure tone audiometry reveals profound sensorineural hearing loss or deafness on the right side. △, air conduction on right side; x, air conduction on left side; -, bone conduction on right side; >, bone conduction on left side; □ △ ○, no response. Hearing levels in dBHL of the arrow markings are insignificant Fig. 2 Initial diffusion weighted imaging (a) and follow up imaging (b) showed hyperintensity mainly on right middle cerebellar peduncle. More extended hyperintense lesion is noted adjacent pontine tegmentum and anterior inferior cerebellum a b Neurol Sci (2008) 29:359–362 361 Fig. 3 Transfemoral cerebral angiography depicting both posterior inferior cerebellar artery (arrowhead) and left anterior inferior cerebellar artery (AICA, arrow). Right AICA is poorly delineated. Left panel; early phase, Right panel; delayed phase wave formation to a 80 dB rarefaction monoaural click stimuli on the right side. On the fifth admission day, the patient’s gait ataxia became aggravated and a follow-up DWI depicted a further hyperintense lesion on the ipsilateral anterior inferior cerebellum (Fig. 2b). A blink reflex test and facial nerve conduction study demonstrated a right pontine origin lesion (i.e., no wave formation of right R1 and R2, and delayed terminal latency with low compound muscle action potential in the ipsilateral facial nerve). An electrocardiography and transthoracic echocardiography revealed no abnormalities. A transfemoral cerebral angiography (TFCA) showed faint visualization of right AICA, suggesting in situ thrombosis (Fig. 3). Six weeks later, the patient’s vertigo and ataxia improved; however, profound hearing loss, facial palsy, and hypaesthesia remained. She was discharged and is presently treated with secondary stroke prevention therapies. Discussion When a physician encounters a patient with dizziness, if the dizziness is vertigo in nature, it is important to consider whether it is of central or peripheral origin. Most instances of central vertigo are usually accompanied by brainstem or cerebellar signs (sensory impairment or ataxia). In a case of central vertigo, something physicians should not overlook is AICA infarction. Because the internal auditory artery (labyrinthine artery), a branch of the AICA, supplies the peripheral auditory system − including the inner ear − AICA occlusion can cause sudden hearing loss and vertigo, with no other neurological deficits [11, 12]. The cochlear origin is supported by the fact that the inner ear lacks collateral blood flow and has a high metabolic rate [7, 11, 12]. Experimental studies of transient internal auditory artery occlusion have revealed that the cochlea is more sensitive to ischaemia than the vestibular labyrinth [13, 14]. Our patient also showed inner ear dysfunction as a prodromal sign, before other neurological signs, as found in most previous reports [7–10]. It is of concern that our case involves recurrent hearing loss with vertigo, which prompts physicians to make a tentative diagnosis of MD until such time as audiovestibular examination and neuroimaging are performed. In our patient, right AICA was not well delineated on TFCA. Although unilateral AICA is sometimes not well visualized as a normal variant with dominance in the contralateral side, the reason as to why our patient initially presented as MD seems to result from recurrent low flow by in situ thrombosis of AICA, which was followed by a progression to AICA orifice occlusion and resulted in a typical AICA stroke. There was no evidence of cardio-embolic risk factor. It is plausible that initial acute auditory syndrome and delayed appearance of other neurological deficits in our patient may also be explained by the selective vulnerability of the cochlea to ischaemia. Although MD is not infrequent in the elderly [5] and our case appears to be similar to MD, it should be noted that several points are incompatible with MD. First, although the patient did not perform an audiogram in the phase when she was having episodic hearing loss with vertigo before admission, profound hearing loss would be unusual in typical MD, especially within the few months following the symptom onset that suggested MD. Although not diagnostically admitted, the BAER finding, which showed absent waves in the right side, might result from labyrinthine ischaemia and demonstrates the degree of ischaemic damage below the cochlear. Second, the first audiovestibular loss, developed one month previous and lasting several minutes, is usually suggestive of vascular origin and is not typical of MD. Previous studies have reported that episodic audiovestibular symptoms before typical AICA stroke were brief, lasting only minutes [8]; such brief durations can warn of an impending brainstem stroke [15]. Our patient provides an edification that if elderly patients with vascular risk factors initially present with isolated recurrent vertigo, together with deafness lasting a brief duration, before revealing additional brainstem or cerebellar signs, physicians may also consider the possibility of an impending symptom of AICA infarction 362 before assessing diagnostic tests such as MR imaging, nystagmography, or pure tone audiometry. Conflict of Interest statement All Authors have no financial disclosures. References 1. Baloh RW (1998) Dizziness, hearing loss, and tinnitus. Philadelphia, FA Davis 2. 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