Hearing Loss, Imbalance, and Diplopia in a 44-Year-Old Man

A 44-year-old man sought care for new right-sided tinnitus and sensorineural hearing loss. He was treated with high-dose oral prednisone and acyclovir. Later, mild, intermittent dizziness developed, which progressed to constant, moderate dizziness and was exacerbated by sudden head movements. He participated in vestibular rehabilitation with only mild improvement. Within 5 months of tinnitus onset, horizontal binocular diplopia also developed. Examination showed spontaneous left-beating torsional nystagmus in primary gaze, down-beating nystagmus with leftward gaze, and right-beating torsional nystagmus in rightward gaze. Head impulse testing to the right produced a catch-up saccade. Dix-Hallpike maneuver in both positions led to leftward torsional nystagmus followed by down-beating nystagmus. He had full range of eye motion. There was evidence of asymmetric hearing loss on the right and moderate gait unsteadiness; he was able to complete only a few steps in tandem. Neurologic examination findings were otherwise normal. Oculomotor testing demonstrated abnormalities supportive of a central nervous system disorder. These included excessive square-wave jerks, impaired smooth pursuit, and direction-changing nystagmus. Results of cerebrospinal fluid studies included a normal opening pressure, pleocytosis, and increased protein concentration. Serum and cerebrospinal fluid paraneoplastic evaluations showed a unique immunofluorescence staining pattern on rodent brain tissue by patient immunoglobulin G, which was later confirmed to be immunoglobulin G antibodies to kelch like family member 11. Whole-body positron emission tomography showed a single anterior mediastinal mass, which was then resected. The patient was diagnosed with a paraneoplastic anti-kelch like family member 11 rhombencephalitis with an extratesticular seminoma. After removal of the mediastinal mass, intravenous methylprednisolone was started. The patient had symptom stabilization but no clinical improvement. Cyclophosphamide was added to the weekly pulse-dose intravenous corticosteroids. He had mild improvement in vertigo and gait imbalance. For symptomatic management of the vertigo, he received baclofen, citalopram, and vestibular rehabilitation. He continued to have slow improvement. After approximately 1 year of cyclophosphamide treatment, his gait normalized and nystagmus diminished, although he had persistent neurologic deficits including spontaneous down-beating nystagmus and a few intermittent square-wave jerks. The intravenous methylprednisolone infusions were tapered, with continued examination stability. After stable symptoms and examination findings, cyclophosphamide was discontinued. After discontinuation of cyclophosphamide, new central sensorineural hearing loss developed suddenly in his left ear. This improved with additional intravenous methylprednisolone treatment. Mycophenolate mofetil was also started, and corticosteroids were tapered. Repeated positron emission tomography of the body showed no recurrence of seminoma. Symptoms and audiography findings were stable after 10 months, so the patient elected to discontinue immunosuppression again and has remained stable. Kelch like family member 11 autoimmunity is a distinct paraneoplastic syndrome associated with encephalitis and testicular germ cell tumors (including seminoma).

Unilateral Multicanalicular Vestibular Lithiasis of Right Horizontal and Posterior Semicircular Canals: An Unusual Case

Vestibular lithiasis (canalolithiasis as well as cupulolithiasis) commonly exists in monocanalicular forms involving one of the three semicircular canals, frequent posterior, less frequent horizontal, and very rarely anterior. It is treated with canal clearing maneuvers intended to reposition the otoconia from the semicircular canal (where they have inappropriately entered) through the utricular exit in the nonampullary arm of the semicircular canal to the utricle (where they normally remain as a part of utricular gelatinous matrix). The cases of multicanalicular vestibular lithiasis with the involvement of more than one semicircular canal require meticulous identification of the involved canals and multiple different canal-clearing maneuvers for effective treatment. A 70-year-old male patient with no significant history of previous medical or otologic illnesses or head trauma presented with a 1-day history of vertigo with positional aggravation. A one-time performed diagnostic supine head roll test elicited three different patterns of positional nystagmus, each with an accurate localizing and lateralizing value. Diagnosis of unilateral multicanalicular vestibular lithiasis of right horizontal and posterior semicircular canals was entertained based on the pattern of the elicited positional nystagmi on the supine roll test. The upbeating torsional nystagmus that localizes the involvement to the posterior semicircular canal was paradoxically elicited by supine head roll test and not by the Dix–Hallpike test. As horizontal semicircular canalolithiasis causes severe symptoms, its treatment preceded that of concurrent posterior semicircular canalolithiasis. The patient was successfully treated with multiple sessions of canalith repositioning maneuvers (CRMs) spread over 24 hours. It is important to perform both positional tests, namely Dix–Hallpike maneuver, and supine head roll test, in cases suspected to have multicanalicular vestibular lithiasis. The positionings may need to be repeated several times to unveil multiple nystagmi, each with different localizing and lateralizing values. Identifying treatment priorities with CRM for the individual semicircular canals is crucial, and the canal that is liable to cause severe symptoms needs early clearance of the otoconial debris. If a CRM fails to clear a semicircular canal, an alternative maneuver may need to be executed. Clinicians involved in the care of cases with multicanalicular vestibular lithiasis should be well versed with all possible backup maneuvers for clearing each of the three semicircular canals.

Pulse-synchronous torsional nystagmus

Purely torsional spontaneous nystagmus almost always has a central vestibular cause. We describe a man with spontaneous pulse-synchronous torsional nystagmus in which the clockwise component corresponded to his pulse upswing, in keeping with a peripheral vestibular cause; following imaging we diagnosed left-sided superior canal dehiscence syndrome. Identifying pulse synchronicity of spontaneous nystagmus may help to distinguish central from peripheral vestibular torsional nystagmus, and is readily confirmed at the bedside using Frenzel’s glasses and a pulse oximeter.

Clinical and VNG Features in Anterior Canal BPPV—An Analysis of 13 Cases

Objective: To define diagnostic VNG features in anterior canal BPPV during positional testing (Dix-Hallpike, supine head hanging, and McClure Pagnini tests).Study Design: A retrospective study of patients diagnosed with anterior canal BPPV across four referral centers in New Delhi, Kochi, Bangalore, and Dubai.Subjects and Methods: Clinical records of 13 patients with AC BPPV out of 1,350 cases, during a 3-years period, were reviewed and analyzed by four specialists.Results: Four patients had positional down beating nystagmus with symptoms of vertigo during the bilateral DHP maneuver. Seven cases had positional down beating nystagmus only on one side of DHP. Typical down beating nystagmus was seen in 10 out of 13 cases during the straight head hanging maneuver. Down beating torsional nystagmus was seen in 6 out of 13 cases. Down beating with horizontal nystagmus was seen in three cases (in DHP and MCP mainly) while pure down beating nystagmus during SHH was only seen in four cases.Conclusion: We conclude that anterior canal BPPV is a rare but definite entity. It may not be apparent on positional testing the first time, so repeated testing may be needed. The most consistent diagnostic maneuver is SHH though there were patients in which findings could only be elicited using DHP testing. We recommend a testing protocol that includes DHP testing on both sides and SHH. MCP testing may also evoke DBN with or without the torsional component. Reversal of nystagmus on reversal of testing position is unusual but can occur. The Yacovino maneuver is effective in resolving AC BPPV. We also propose a hypothesis that explains why DHP testing is sensitive to AC BPPV on either side, whereas MCP lateral position on one side is only sensitive to AC BPPV on one side. We have explained a possible role for the McClure Pagnini test in side determination and therapeutic implications.