A 61-year-old female patient was admitted to the emergency unit with the complaint of 2 days' dizziness of whirling nature accompanied by nausea and vomiting. The patient had medical history of hypertension and diabetes mellitus. Bedside neurologic examination revealed otherwise nonspecific. Brain diffusion weighted image (DWI) for the differential diagnosis of a central pathology was normal. On otoscopic examinations, normal right tympanic membrane was seen, but purulent ear discharge was filling the left external ear canal. The patient had been feeling a loss of hearing in her left ear for quite a long time and had been visiting a local otorhinolaryngologic clinic for several months diagnosed and treated for chronic external otitis in the left ear. The patient showed spontaneous, right-beating horizontal nystagmus (6.36 d/s). There was no gaze-evoked nystagmus, and head thrust test was normal. Left-beating horizontal nystagmus (9.70 d/s) was noted after head shaking. Dix-Hallpike test to either side provoked geotropic horizontal nystagmus, but the nystagmus was stronger on the left side (21.23 d/s on the left side compared to 15.00 d/s on the right) and so was the degree of symptoms. Geotropic direction-changing horizontal nystagmus was demonstrated on both sides during the supine roll test. The intensity of the nystagmus on both sides was comparable showing 17.71 d/s on the right side and 17.03 d/s on the left side. The latency and duration of positional nystagmus were 2 and 29 seconds, respectively on the right side, and 3 and 30 seconds, respectively on the left side. On the head bending nystagmus or lying-down nystagmus test, left-beating horizontal nystagmus (7.00 d/s) on the head bending nystagmus test and right-beating horizontal nystagmus (14.93 d/s) on the lying-down nystagmus test was demonstrated, respectively (). The bithermal caloric test response demonstrated unilateral canal paresis of 29.98% on the left side. These findings collectively led to the initial impression of geotropic variant of the left lateral semicircular canal BPPV on the left side combined with ipsilesional external otitis. The patient received the modified Lempert maneuver for treatment of the presumptive BPPV and was prescribed with empirical topical and oral antibiotics for management of the external otitis. She was discharged from the emergency unit for an our clinic visit scheduled for the following day.\nAt the next follow-up visit, the patient still complained of a positional vertigo aggravated on a leftward head turn, despite the therapeutic maneuver. A neurootologic examination revealed weak spontaneous, right-beating horizontal nystagmus. After head shaking, the horizontal nystagmus was seen to beat to the left. The patient was negative for positional nystagmus from the Dix-Hallpike test and supine roll test, but left-beating horizontal nystagmus on the head bending nystagmus test and right-beating horizontal nystagmus on the lying-down nystagmus test was demonstrated, respectively. From these atypical nystagmus patterns, a thorough examination of the ear was undertaken, where keratinous debris and inflammatory polyps were hidden behind the purulent material blocking the left external auditory canal. The keratinous debris was cleared and polypoid tissues were removed for tissue pathology, after which the patient was sent for the TBCT. On the TBCT, the left middle ear cavity was mostly occupied with soft tissue eroding all the ossicles as well as the bone overlying the lateral semicircular canal and the tegmen tympani (). Magnetic resonance imaging and brain DWI were taken but reported negative for intradural intracranial invasion.\nUnder the diagnosis of left cholesteatomatous chronic otitis media with the complication of labyrinthine fistula, the patient was admitted to the hospital. The patient was instructed an absolute bed rest in a 30 degree head elevated position. A brain-blood-barrier-penetrating intravenous antibiotic and osmotic laxative were prescribed. Pure tone audiometry, taken on the day of admission, showed normal air-conduction threshold of 18 dB HL for the right ear, and a mixed hearing loss with an air-conduction threshold of 78 dB HL for the left ear (). Tissue pathology taken on the first clinic exam was reported as a cholesteatoma. After resolution of dizziness, canal wall-down tympanomastoidectomy was performed for complete removal of the cholesteatoma on the 7th day of admission. Intraoperatively, dehiscence of the bony labyrinth of the lateral semicircular canal was observed without any leakage of perilymphatic fluid after the removal of the matrix. The dehiscence was covered by bone paste and muscle fascia. In the immediate postoperative period, the patient complained of dizziness, and a weak right-beating horizontal nystagmus was observed. Otherwise, it was uneventful postoperatively, free from signs of facial palsy or cerebrospinal fluid leaks. One day after the surgery, dizziness was subsided and there was no evidence of spontaneous nystagmus. The patient was discharged from the hospital on the 8th postoperative day without any evidence of complications. On follow up 3 months postoperatively, the patient was free of dizziness and felt improved hearing, of which pure tone audiogram showed improved hearing thresholds (left air-conduction threshold of 45 dB and bone-conduction threshold of 23 dB) ().
Patient 1. A 25-year-old man presented to the emergency room with an intracranial complication secondary to the chronic otitis media as shown in a CT scan (). He was diagnosed and treated when neurologically stable. In this case the vestibular damage has occurred progressively over many years and as such was successfully compensated. The affected ear was the left; the fistula sign was negative (no nystagmus under rubber bulb applied pressure to the external ear) but the bedside head impulse test was positive due to the appearance of intense refixation saccades that indicated a major vestibular deficit.