Pearls &amp; Oy-sters: A rare presentation of chronic intracranial hypertension with concurrent deafness and blindness

Hosseini, Mersedeh Bahr; McGuire, Laura Stone; Stosic, Milena; Moss, Heather E.; Carrithers, Michael D.

doi:10.1212/wnl.0000000000002868

Cited by 8 publications

(8 citation statements)

References 6 publications

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“…[ 118 ] Inversion of ABR waveforms in response to a change in stimulus polarity and absence of ABR recordings has also been reported in case reports. [ 111 112 ] These findings improved or normalized as ICP returned to normal.…”

Section: Atypical Presentationsmentioning

confidence: 97%

“…Hearing loss as a prominent presenting feature of IIH has been reported in five patients [ Table 6 ]. [ 110 111 112 113 114 ] Four patients presented with bilateral sensorineural hearing loss, and one had unilateral conductive hearing loss. [ 113 ] The diagnosis of IIH was questionable in four patients, due to the absence of papilledema or insufficient imaging to exclude venous sinus thrombosis.…”

Section: Atypical Presentationsmentioning

confidence: 99%

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Atypical presentations of idiopathic intracranial hypertension

Chen

Newman

Biousse

2021

Taiwan Journal of Ophthalmology

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Idiopathic intracranial hypertension (IIH) is a disorder of unknown etiology that results in isolated raised intracranial pressure. Classic symptoms and signs of IIH include headache, papilledema, diplopia from sixth nerve palsy and divergence insufficiency, and pulsatile tinnitus. Atypical presentations include: (1) highly asymmetric or even unilateral papilledema, and IIH without papilledema; (2) ocular motor disturbances from third nerve palsy, fourth nerve palsy, internuclear ophthalmoplegia, diffuse ophthalmoplegia, and skew deviation; (3) olfactory dysfunction; (4) trigeminal nerve dysfunction; (5) facial nerve dysfunction; (6) hearing loss and vestibular dysfunction; (7) lower cranial nerve dysfunction including deviated uvula, torticollis, and tongue weakness; (8) spontaneous skull base cerebrospinal fluid leak; and (9) seizures. Although atypical findings should raise a red flag and prompt further investigation for an alternative etiology, clinicians should be familiar with these unusual presentations.

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Section: Atypical Presentationsmentioning

confidence: 97%

Section: Atypical Presentationsmentioning

confidence: 99%

Atypical presentations of idiopathic intracranial hypertension

Chen

Newman

Biousse

2021

Taiwan Journal of Ophthalmology

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“…Extravasation is enhanced by a reflex increase of the ophthalmic artery pressure. Vision loss is usually transient but may be persistent if bleeding occurs within the optic nerve ( 19 , 20 ). Large pressure waves may cause direct injury to pial bridging veins and render the posterior optic nerve ischemic.…”

Section: Effect Of Increased Subarachnoid Pressurementioning

confidence: 99%

“…In addition, shearing and normal forces may cause dysfunction of the vestibuloacoustic nerve. Tinnitus, hearing loss, or equilibrium disturbances may be transient, but large pressure waves are likely to cause permanent loss of cochlear and vestibular function ( 19 ).…”

Section: Effect Of Increased Subarachnoid Pressurementioning

confidence: 99%

Pressure-Volume Relationships in the Spinal Canal and Potential Neurological Complications After Epidural Fluid Injections

Bosscher

2022

Front. Pain Res.

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High-volume fluid injections into the spinal canal may lead to severe neurological complications. But when anatomical or pathological conditions in the spinal canal are unfavorable, even small volume epidural injections can cause dangerously high epidural, subarachnoid, and intracranial pressures or pressure gradients. Data obtained from the scientific literature and direct clinical observation are used to derive a first-order approximation of epidural, subarachnoid, and intracranial pressure responses to epidural fluid injections. Maximum allowable fluid volumes for single or multiple divided fluid injections over time are calculated. In the presence of spinal pathology, 10 ml of fluids may increase epidural pressure to >100 mmHg. Injection speed >4 ml per second may also generate dangerously high intraspinal and intracranial pressures. Intermitted bolus injections provide limited protection, but intraspinal pressures may rise very fast when a critical total injected volume is reached. Potential complications of increased intracranial pressures or large pressure waves include nerve palsies, tinnitus, blindness, stroke, and death. Spinal injections or endoscopy should be performed in an awake responsive patient or with direct cerebrospinal fluid pressure monitoring. A set of guidelines for epidural fluid management is given.

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“…Hearing symptoms caused by ICH are generally reversible. However, neurological outcomes can sometimes be worse if therapy is not started promptly . As children seldom complain of tinnitus spontaneously, careful observation of pediatric APL patients is warranted to avoid irreversible neurological damage due to prolonged mild ICH.…”

mentioning

confidence: 99%