Tinnitus update: what can be done for the ringing?

Physicians will frequently encounter patients who report tinnitus. Tinnitus is a symptom whereby there is the perception of sound or sounds in the ear or head in the absence of an external source of sound. Most individuals experiencing tinnitus will have a neutral reaction to the percept, but in a small proportion of patients, tinnitus can be a debilitating symptom. When it causes burden, patients can be affected in multiple different facets of life, including impairment in sleep, hearing cognition and psychological and psychiatric well-being, often resulting in high healthcare utilisation and societal costs. Hence, chronic, disabling tinnitus is a complex condition with multifactorial causes and multiple perpetuating biopsychosocial factors. Despite efforts to increase knowledge about its pathophysiology and research into treatments, little impact on real-world clinical practice has been seen. There are no proven effective pharmacological treatments or complementary medicines specifically for chronic, disabling tinnitus. Despite this, there is a role for treating this condition through a multidisciplinary approach specifically targeting comorbid active psychiatric conditions, using hearing aids in appropriate clinical settings such as in those with a coassociated confirmed hearing loss, and specialised cognitive behavioural therapy for patients reporting bothersome tinnitus. Cognitive behavioural therapy remains the most valuable evidence-based intervention in this regard. This narrative review attempts to summarise the current understanding in terms of pathophysiology, assessment and treatment of tinnitus for the internal physician who may encounter patients with disabling, chronic tinnitus.

Practical approach to vertigo: a synthesis of the emerging evidence.

Vestibular presentations are common in both the acute and recurrent setting, burdening emergency departments and community clinics alike. Commonly, an unease among the emergency or general physician is felt, and historically focus has been on gaining knowledge of each potential disease rather than honing the diagnostic process. Consequently, this paper focuses on the approach itself, helping to categorise this common complaint into one of four main syndromes: the Acute Vestibular Syndrome, Recurrent Positional Vertigo, Recurrent Spontaneous Vertigo, and Imbalance. Its simplicity is aimed to minimise uncertainty and highlight clear scenarios when to refer. Together with descriptions of the clinically relevant pathophysiology, the reader should approach the vertiginous patient with a new clarity.

Acute evaluation of the acute vestibular syndrome: differentiating posterior circulation stroke from acute peripheral vestibulopathies.

This review article aims to provide an evidence-based approach to evaluating the patient who presents with acute prolonged, spontaneous vertigo in the context of the acute vestibular syndrome (AVS). Differentiation of posterior circulation stroke (PCS) presenting as an AVS has been regarded as an important diagnostic challenge for physicians involved in acute care. Current evidence suggests that a targeted approach to history taking and physical examination with emphasis on the oculomotor examination, more specifically the HINTS (Head Impulse/Nystagmus/Test-of-skew) examination battery, yields a higher sensitivity for the diagnosis of PCS than even standard magnetic resonance imaging with diffusion-weighted imaging. However, most studies have only validated the utility of the HINTS examination when performed by experts, who interpret the most powerful component of HINTS, namely the head impulse test (HIT), considerably different to the novice. Several investigations useful in the differentiation of the AVS are becoming more accessible and portable, such as videooculography with Frenzel goggles and video head impulse testing (vHIT), which allows for the quantitative assessment of the HIT. In clinical practice, vHIT has already become accepted as standard of care in the evaluation of AVS.

Fatal ischemic stroke complicating acute multifocal placoid pigment epitheliopathy: histopathological findings.

A previously healthy 29-year-old man was admitted to a tertiary referral center with acute left hemiparesis followed shortly by de novo convulsive status epilepticus. This was in the context of a 2-month history of flu-like symptoms, severe headaches, and retinopathy recently diagnosed as acute multifocal placoid pigment epitheliopathy. Neuroimaging demonstrated bilateral, multiple territory cerebral infarction. Despite intravenous methylprednisolone and craniotomy for the management of raised intracranial pressure, the patient deteriorated and died 14 days later. At autopsy, multiple infarcts of varying ages within a 10-day period were seen in association with a segmental giant cell vasculopathy of meningeal arteries.

Sudden unilateral audiovestibular loss due to acute labyrinthine haemorrhage can be missed on early MRI brain sequences: case report.

Background: Labyrinthine haemorrhage is a rare vascular disorder often presenting with the triad of acute vertigo, sudden sensorineural hearing loss and tinnitus. There are minimal reports on imaging progression over the acute period. Index case: A woman in her mid-40s presented with acute vertigo, sudden left-sided hearing loss and tinnitus, consistent with acute unilateral audiovestibular loss. Left peripheral vestibular hypofunction was confirmed acutely on video head impulse testing, and pure tone audiometry showed a profound left sensorineural hearing loss. An MRI brain including diffusion-weighted imaging within 24 hours was normal. Delayed MRI brain and internal acoustic canal after 7 days demonstrated increased 3D fluid-attenuated inversion recovery and T1 signal throughout the left cochlea and semicircular canals, without contrast enhancement. This was consistent with labyrinthine haemorrhage. She received early oral prednisone followed by three doses of intratympanic dexamethasone. At 12 months follow-up the patient remained profoundly deaf, however, balance and vestibular symptoms improved with early vestibular physical rehabilitation. Conclusion: We report a case of acute labyrinthine haemorrhage missed on an early MRI brain sequence. This diagnosis should be considered in presentations of acute audiovestibular loss, and delayed MRI including internal auditory canal sequences may be important for diagnosis.

Right ventricular thrombus detection and multimodality imaging using contrast echocardiography and cardiac magnetic resonance imaging.

We present the case of right ventricular thrombus formation associated with a right ventricular infarct secondary to a proximal right coronary artery thrombus, which was not evident on transthoracic echocardiography but detected on both delayed gadolinium enhanced magnetic resonance imaging and microsphere contrast echocardiography. The diagnosis of right ventricular thrombosis altered the decision to place an implantable cardiac defibrillator in this patient. Anticoagulation with warfarin resulted in resolution of the thrombus. This case highlights the utility of multimodality imaging in the detection and follow-up of right ventricular thrombus in the setting of right ventricular myocardial infarction, and the effectiveness of anticoagulation therapy.

Multiple sclerosis: Diagnosis, disease-modifying therapy and prognosis.

BACKGROUND: Multiple sclerosis (MS) is a multifocal inflammatory central nervous system disorder. There are now many highly effective disease-modifying therapies (DMTs) available as treatment options, which have a significant impact on disease activity and long-term disability. OBJECTIVE: The aim of this article is to provide a concise overview of the diagnosis, DMTs and prognosis of MS. DISCUSSION: The diagnosis of MS is made on clinicoradiological grounds to prove dissemination of disease in both time and space in the nervous system. While the expanding options of DMTs have had a significant impact on disability, they make medication selection for individual patients more complicated. Patients with MS often have a model of care shared between the neurologist and the general practitioner. This review article summarises the key aspects of the diagnosis, DMTs and prognosis of MS relevant to the general practitioner.

Multiple sclerosis- diagnosis, management and prognosis.

BACKGROUND: Multiple sclerosis is the most common chronic disabling disease of the central nervous system in young adults. OBJECTIVE: This article summarises the diagnosis, management and prognosis of multiple sclerosis. DISCUSSION: Multiple sclerosis usually starts with an acute episode of neurological disturbance, termed a 'clinically isolated syndrome', followed by an illness phase punctuated by relapses and remissions which may transition after 10 years to a phase of progressive accumulation of disability without relapses. Fifteen to 20% of patients will have a progressive course from the onset. There is significant interpatient variability in prognosis. The main diagnostic criteria are clinical, supported by investigations including magnetic resonance imaging and lumbar puncture and evoked potentials. First line disease modifying agents for relapsing remitting multiple sclerosis include interferon-ß and glatiramer. First line treatment for relapses is usually intravenous methylprednisolone for 3 days. Troublesome symptoms may include spasticity, parasthesias, tremor, erectile dysfunction, depression and anxiety, fatigue and pain. After excluding differential diagnoses, symptomatic management includes pharmacological agents, allied health consultation and continence strategies. Although pregnancy reduces disease activity, there is a higher risk of relapse in the postpartum period.

An Australian State-Based Cohort Study of Autoimmune Encephalitis Cases Detailing Clinical Presentation, Investigation Results, and Response to Therapy.

Introduction: Autoimmune encephalitis is a disorder associated with antibodies directed against central nervous system proteins with variable clinical features. This study aims to add to knowledge of the disease by reporting the details of a cohort of patients with autoimmune encephalitis in Queensland, Australia. Methodology: We surveyed patients with autoimmune encephalitis diagnosed and managed through public hospitals in Queensland, Australia between 2010 and the end of 2019. Cases were identified via case detection through a centralized diagnostic neuroimmunology laboratory (Division of Immunology, HSQ Pathology Queensland Central Laboratory, Brisbane, Queensland, Australia) and a survey of neurologists. Data including demographic details, clinical presentation, investigation results, treatments including immune therapy and outcomes was collected. Results: Sixty cases of antibody positive autoimmune encephalitis were identified. Twenty-eight were of anti-NMDA-receptor encephalitis with other cases associated with antibodies against LGi1, Caspr2, glycine receptor, DPPX, GABAB receptor, IgLON5, GFAP, and SOX1. The number of diagnosed cases, especially of anti-NMDA-receptor encephalitis has markedly increased over the period 2017 to 2019. Clinical presentations were marked by heterogeneous symptom complexes and prolonged hospital admissions. Imaging studies were largely normal or non-specific. There was a response to immune therapy and a low mortality rate. Most cases affected by this disorder were left with ongoing symptoms associated with mild disability. Conclusion: Autoimmune encephalitis in Queensland, Australia is an increasingly common but complex clinical entity marked by heterogeneous presentations, response to immune therapy and outcome results marked by low mortality and incomplete recovery.

Hirayama disease.

This article discusses three patients with likely Hirayama disease. They have no other significant past medical history and no personal or family history of other neurological disorders. Hirayama disease is a form of cervical myelopathy attributed to forward displacement of the posterior cervical dural sac on neck flexion with resultant cord compression and/or venous congestion. It is characterized by a pure motor focal amyotrophy in the distribution of C7, C8 and T1 spinal segmental-innervated muscles and differs from other motor neuron diseases by virtue of its ultimately non-progressive course.

Mechanisms of upper limb amyotrophy in spinal disorders.

Upper limb amyotrophy may occur as an indirect consequence of various spinal disorders, including ventral longitudinal intraspinal fluid collection, Hirayama disease and high cervical cord compression. We present patients who suffer from each of these and review the literature on the three conditions with emphasis on the pathogenesis of amyotrophy. We propose that pathology some distance from the lower cervical spinal cord may affect normal venous drainage, resulting in venous congestion and reduced perfusion pressure which, in turn, could result in anterior horn cell dysfunction in all three disorders.

Case report of multiple sclerosis diagnosis in an 82-year old male.

An 82-year old male, with no significant past medical history, presented with a subacute right foot drop in the setting of a 14-month history of generalised weakness, highly-responsive to steroids. Temporal artery and vastus lateralis biopsies were normal. Vasculitic screen and inflammatory markers were normal. Lumbar puncture revealed elevated cerebrospinal fluid (CSF) protein without oligoclonal bands. Visual evoked response (VER) was normal. Magnetic resonance imaging (MRI) of his lumbar spine showed compression of exiting L5 nerve root. He had three cerebral MRI scans spaced over the 12 month period, which showed a progressive increase of T2 and fluid attenuated inversion recovery (FLAIR) hyperintense lesions consistent with active demyelinating plaques. He was treated with intravenous methylprednisolone 1g daily for three days with a weaning regimen of oral prednisolone, resulting in a full return of power and a resolution of his right foot drop. He was diagnosed with late-onset multiple sclerosis (LOMS), and was treated with monthly natalizumab. A literature review of LOMS is discussed.

Diffusion weighted imaging with trace diffusion weighted imaging, the apparent diffusion coefficient and exponential images in the diagnosis of spinal cord infarction.

A 73-year-old man, with a history of hypertension and left supraclavicular fossa arteriovenous malformation with multiple previous uncomplicated vessel embolisation procedures, presented with acute spastic quadriparesis and urinary retention following upper limb angiography and embolisation. There was no evidence of preceding infection or neurological disease prior to the event. Cerebrospinal fluid analysis was unremarkable. MRI of the cervical spine with a 1.5 Tesla magnet performed 13 hours from symptom onset revealed bilateral paramedian intramedullary T2-weighted signal change without gadolinium enhancement limited to the grey matter with corresponding diffusion restriction extending from C5-6 down to the mid-T1. The diagnosis of cervical spinal cord infarction (SCI) was made and the patient was given regular aspirin and atorvastatin. On follow-up at 3 months, there was modest improvement with respect to his quadriparesis and was walking unaided. An extensive literature review on the role of MRI in SCI is discussed.

Subacute combined degeneration of the spinal cord despite prophylactic vitamin B12 treatment.

We describe a 35 year-old man presenting with a four-week history of non-painful limb paraesthesias and unsteady gait causing falls. On examination he had an ataxic gait associated with dorsal column sensory loss. He had a medical history of a partial gastrectomy six years prior and anaemia. He had received monthly intramuscular hydroxycobalamin injections since the gastrectomy. Laboratory tests revealed normal vitamin B12 and holotranscobalamin levels, a reduced serum caeruloplasmin of 0.05 g/L (normal: 0.22-0.58 g/L), a copper-to-caeruloplasmin ratio of 1.9 µmol/L (11.0-22.0 µmol/L) and a reduced 24-hour urinary copper concentration of <0.30 µmol/L (0-0.3 µmol/L). Cerebrospinal fluid analysis, nerve conduction studies, electromyography and visual-evoked responses were unremarkable. MRI revealed abnormal hyperintense signal in the cervical dorsal columns. Hypocupric myelopathy was diagnosed and he was treated with daily oral elemental copper. Three months later, his walking and balance had improved although there was no change noted on MRI.

A patient with steroid responsive encephalopathy associated with autoimmune thyroiditis.

We present a 58-year-old female with gradual cognitive decline and gait instability over 6 months. Her motor examination was notable for myoclonus, brisk reflexes with flexor plantar responses, and a cautious gait without ataxia. Cognitive testing revealed mildly impaired attention, but profoundly impaired calculation, judgment and visual memory. There were no manifestations of autoimmune thyroid disease. Routine laboratory analysis was unrevealing. Cerebrospinal fluid analysis was remarkable only for an elevated protein of 0.64 g/L (normal <0.45 g/L). Electroencephalography demonstrated intermittent bitemporal slowing. Brain MRI with gadolinium demonstrated extensive bilateral subcortical and periventricular white matter T2-weighted and hyperintensity on fluid attenuated inversion recovery MRI. Elevated anti-thyroperoxidase antibody of 8.07 IU/mL (<5.61 IU/mL) and thyroglobin antibody of 9.85 IU/mL (<4.11 IU/mL) were found and steroid responsive encephalopathy associated with autoimmune thyroiditis was diagnosed. Methylprednisolone (1 g daily for 3 days) resulted in dramatic improvement in cognition and mobility, which remained on follow-up.