No abstract

BACKGROUND AND PURPOSE:No reliable MR imaging marker for the diagnosis of Menière disease has been reported. Our aim was to investigate whether the obliteration of the inferior portion of the vestibule and the contact with the stapes footplate by the vestibular endolymphatic space are reliable MR imaging markers in the diagnosis of Menière disease. MATERIALS AND METHODS:We retrospectively enrolled 49 patients, 24 affected by unilateral sudden hearing loss and 25 affected by definite Menière disease, who had undergone a 4-hour delayed 3D-FLAIR sequence. Two readers analyzed the MR images investigating whether the vestibular endolymphatic space bulged in the third inferior portion of the vestibule contacting the stapes footplate. This sign was defined as the vestibular endolymphatic space contacting the oval window. RESULTS:We analyzed 98 ears: 27 affected by Menière disease, 24 affected by sudden sensorineural hearing loss, and 47 that were healthy. The vestibular endolymphatic space contacting the oval window showed an almost perfect interobserver agreement (Cohen ϭ 0.87; 95% CI, 0.69 -1). The vestibular endolymphatic space contacting oval window showed the following: sensitivity ϭ 81%, specificity ϭ 96%, positive predictive value ϭ 88%, and negative predictive value ϭ 93% in differentiating Menière disease ears from other ears. The vestibular endolymphatic space contacting the oval window showed the following: sensitivity ϭ 81%, specificity ϭ 96%, positive predictive value ϭ 96%, negative predictive value ϭ 82% in differentiating Menière disease ears from sudden sensorineural hearing loss ears. CONCLUSIONS:The vestibular endolymphatic space contacting the oval window has high specificity and positive predictive value in differentiating Menière disease ears from other ears, thus resulting in a valid tool for ruling in Menière disease in patients with mimicking symptoms.ABBREVIATIONS: AAO-HNS ϭ American Academy of Otolaryngology-Head and Neck Surgery; BLB ϭ blood-labyrinthine barrier; CH ϭ cochlear hydrops; HC ϭ healthy control; MD ϭ Menière disease; nMD ϭ normal in a patient with MD; NPV ϭ negative predictive value; PPV ϭ positive predictive value; SSHL ϭ sudden sensorineural hearing loss; VEH ϭ vestibular endolymphatic hydrops; VES ϭ vestibular endolymphatic space; VESCO ϭ vestibular endolymphatic space contacting the oval window

Although individual cases of inherited metabolic disorders are rare, overall they account for a substantial number of disorders affecting the central nervous system. Organic acidemias and aminoacidopathies include a variety of inborn errors of metabolism that are caused by defects in the intermediary metabolic pathways of carbohydrates, amino acids, and fatty acid oxidation. These defects can lead to the abnormal accumulation of organic acids and amino acids in multiple organs, including the brain. Early diagnosis is mandatory to initiate therapy and prevent permanent long-term neurologic impairments or death. Neuroimaging findings can be nonspecific, and metabolism- and genetics-based laboratory investigations are needed to confirm the diagnosis. However, neuroimaging has a key role in guiding the diagnostic workup. The findings at conventional and advanced magnetic resonance imaging may suggest the correct diagnosis, help narrow the differential diagnosis, and consequently facilitate early initiation of targeted metabolism- and genetics-based laboratory investigations and treatment. Neuroimaging may be especially helpful for distinguishing organic acidemias and aminoacidopathies from other more common diseases with similar manifestations, such as hypoxic-ischemic injury and neonatal sepsis. Therefore, it is important that radiologists, neuroradiologists, pediatric neuroradiologists, and clinicians are familiar with the neuroimaging findings of organic acidemias and aminoacidopathies. RSNA, 2018.

No abstract

No abstract